Merrill D. Benson
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Merrill D. Benson's Degrees
- PhD Neuroscience University of California, Berkeley
- Doctorate Medicine University of California, San Francisco
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(Suggest an Edit or Addition)Merrill D. Benson's Published Works
Number of citations in a given year to any of this author's works
Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author
Published Works
- A mutation in the amyloid precursor protein associated with hereditary Alzheimer's disease. (1991) (1150)
- Inotersen Treatment for Patients with Hereditary Transthyretin Amyloidosis (2018) (553)
- Apolipoprotein E ∈4 allele distributions in late-onset Alzheimer's disease and in other amyloid-forming diseases (1993) (495)
- Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines (2016) (468)
- Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis (2014) (437)
- The molecular biology and clinical features of amyloid neuropathy (2007) (410)
- Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee (2018) (370)
- A primer of amyloid nomenclature (2007) (344)
- Amyloid: Toward terminology clarification Report from the Nomenclature Committee of the International Society of Amyloidosis (2005) (339)
- Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis (2010) (337)
- The protofilament substructure of amyloid fibrils. (2000) (333)
- Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis. (2015) (321)
- Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis (2012) (294)
- Amyloid Fibril Protein Nomenclature - 2002 (2002) (221)
- Transthyretin: a review from a structural perspective (2001) (220)
- Variant apolipoprotein AI as a major constituent of a human hereditary amyloid. (1988) (215)
- Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee (2020) (204)
- Primary structure of an amyloid prealbumin and its plasma precursor in a heredofamilial polyneuropathy of Swedish origin. (1984) (180)
- Hereditary renal amyloidosis associated with a mutant fibrinogen α–chain (1993) (174)
- The X-ray crystal structure refinements of normal human transthyretin and the amyloidogenic Val-30→Met variant to 1.7-Å resolution (1993) (174)
- Polymorphism of human plasma thyroxine binding prealbumin. (1983) (160)
- Direct sequencing of the gene for Maryland/German familial amyloidotic polyneuropathy type II and genotyping by allele-specific enzymatic amplification. (1989) (159)
- Biochemical and molecular genetic characterization of a new variant prealbumin associated with hereditary amyloidosis. (1986) (159)
- Autocrine induction of collagenase by serum amyloid A-like and beta 2-microglobulin-like proteins. (1989) (158)
- Intracellular Ferritin Accumulation in Neural and Extraneural Tissue Characterizes a Neurodegenerative Disease Associated with a Mutation in the Ferritin Light Polypeptide Gene (2004) (155)
- A mutation in apolipoprotein A-I in the Iowa type of familial amyloidotic polyneuropathy. (1990) (153)
- Leukocyte chemotactic factor 2: A novel renal amyloid protein. (2008) (148)
- Targeted suppression of an amyloidogenic transthyretin with antisense oligonucleotides (2006) (144)
- Characterization of a transthyretin (prealbumin) variant associated with familial amyloidotic polyneuropathy type II (Indiana/Swiss). (1986) (135)
- A new human hereditary amyloidosis: the result of a stop-codon mutation in the apolipoprotein AII gene. (2001) (134)
- Clinical development of an antisense therapy for the treatment of transthyretin-associated polyneuropathy (2012) (133)
- Progression of transthyretin amyloid neuropathy after liver transplantation (2010) (128)
- The hereditary amyloidoses. (2003) (120)
- Familial and sporadic Alzheimer's disease (1996) (117)
- Localization of the human prealbumin gene to chromosome 18. (1985) (115)
- Nonexpression of the human serum amyloid A three (SAA3) gene. (1991) (113)
- Progression of cardiac amyloid deposition in hereditary transthyretin amyloidosis patients after liver transplantation (2007) (110)
- Suppressing transthyretin production in mice, monkeys and humans using 2nd-Generation antisense oligonucleotides (2016) (108)
- Amyloid fibrils containing fragmented ATTR may be the standard fibril composition in ATTR amyloidosis (2013) (104)
- Hereditary fibrinogen A alpha-chain amyloidosis: phenotypic characterization of a systemic disease and the role of liver transplantation. (2010) (104)
- Murine amyloid protein AA in casein-induced experimental amyloidosis. (1977) (103)
- In vivo metabolism of a mutant apolipoprotein, apoA-IIowa, associated with hypoalphalipoproteinemia and hereditary systemic amyloidosis. (1992) (99)
- A prospective evaluation of the transthyretin Ile122 allele frequency in an African-American population (2005) (99)
- A novel lysozyme mutation Phe57Ile associated with hereditary renal amyloidosis. (2003) (99)
- Serum amyloid A protein in amyloidosis, rheumatic, and enoplastic diseases. (1979) (97)
- Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations. (2001) (96)
- Hereditary renal amyloidosis with a novel variant fibrinogen. (1994) (96)
- Hereditary amyloid cardiomyopathy caused by a variant apolipoprotein A1. (1999) (91)
- Characterization of amyloid A protein in human secondary amyloidosis: the predominant deposition of serum amyloid A1. (1995) (91)
- Generalized amyloid in a family of Swedish origin. A study of 426 family members in seven generations of a new kinship with neuropathy, nephropathy, and central nervous system involvement. (1977) (88)
- Liver transplantation and transthyretin amyloidosis (2013) (85)
- Renal amyloidosis with a frame shift mutation in fibrinogen aalpha-chain gene producing a novel amyloid protein. (1997) (85)
- Familial amyloidotic polyneuropathy (1989) (83)
- Kinetics of serum amyloid protein A in casein-induced murine amyloidosis. (1977) (80)
- Hereditary renal amyloidosis associated with a mutant fibrinogen alpha-chain. (1993) (80)
- Human serum amyloid A. Three hepatic mRNAs and the corresponding proteins in one person. (1988) (77)
- A novel apolipoprotein A-1 variant, Arg173Pro, associated with cardiac and cutaneous amyloidosis. (1999) (77)
- Oculoleptomeningeal amyloidosis in a large kindred with a new transthyretin variant Tyr69His (2003) (75)
- A cell culture system for the study of amyloid pathogenesis. Amyloid formation by peritoneal macrophages cultured with recombinant serum amyloid A. (1999) (75)
- Tertiary structure of an amyloid immunoglobulin light chain protein: a proposed model for amyloid fibril formation. (1995) (75)
- Fibrillar assemblage of variable segments of immunoglobulin light chains: an electron microscopic study. (1973) (73)
- Partial amino acid sequence homology between an heredofamilial amyloid protein and human plasma prealbumin. (1981) (73)
- Leptomeningeal amyloid and variant transthyretins. (1996) (72)
- Oculoleptomeningeal amyloidosis associated with a new transthyretin variant Ser64. (1999) (72)
- SAA suppression of immune response in vitro: evidence for an effect on T cell-macrophage interaction. (1982) (71)
- A point mutation in transthyretin increases affinity for thyroxine and produces euthyroid hyperthyroxinemia. (1990) (70)
- Effect of purified protein SAA on immune response in vitro: mechanisms of suppression. (1979) (70)
- The x-ray crystal structure refinements of normal human transthyretin and the amyloidogenic Val-30-->Met variant to 1.7-A resolution. (1993) (68)
- Neuroserpin Mutation S52R Causes Neuroserpin Accumulation in Neurons and Is Associated with Progressive Myoclonus Epilepsy (2000) (68)
- The 1990 Guidelines for Nomenclature and Classification of Amyloid and Amyloidosis (1991) (67)
- Amino acid structures of multiple forms of amyloid-related serum protein SAA from a single individual. (1988) (66)
- Identification of a new hereditary amyloidosis prealbumin variant, Tyr-77, and detection of the gene by DNA analysis. (1988) (65)
- Low cerebrospinal-fluid concentrations of soluble amyloid β-protein precursor in hereditary Alzheimer's disease (1992) (65)
- Hereditary amyloidosis: description of a new American kindred with late onset cardiomyopathy. Appalachian amyloid. (1987) (65)
- Ostertag revisited: The inherited systemic amyloidoses without neuropathy (2005) (64)
- Prealbumin and retinol binding protein serum concentrations in the Indiana type hereditary amyloidosis. (1983) (64)
- Fibril formation from recombinant human serum amyloid A. (1994) (64)
- Nomenclature of amyloid fibril proteins. Report from the meeting of the International Nomenclature Committee on Amyloidosis, August 8-9, 1998. Part 1. (1999) (63)
- Safety and efficacy of a TTR specific antisense oligonucleotide in patients with transthyretin amyloid cardiomyopathy (2017) (63)
- Organ-specific (localized) synthesis of Ig light chain amyloid. (1999) (63)
- A frame shift mutation in the fibrinogen A alpha chain gene in a kindred with renal amyloidosis. (1996) (62)
- A Review of Tafamidis for the Treatment of Transthyretin-Related Amyloidosis (2015) (60)
- Retrospective study of a TTR FAP cohort to modify NIS+7 for therapeutic trials (2014) (59)
- Orthotopic liver transplantation for hereditary fibrinogen amyloidosis. (2003) (57)
- ISOLATION AND IDENTIFICATION BY SEQUENCE ANALYSIS OF EXPERIMENTALLY INDUCED GUINEA PIG AMYLOID FIBRILS (1974) (56)
- Amyloid seeding of transthyretin by ex vivo cardiac fibrils and its inhibition (2018) (56)
- X-ray crystal structure of the Ala-109-->Thr variant of human transthyretin which produces euthyroid hyperthyroxinemia. (1993) (55)
- An allele of serum amyloid A1 associated with amyloidosis in both Japanese and Caucasians (2003) (55)
- Protein aging hypothesis of Alzheimer disease (2000) (55)
- Methylprednisolone pulse therapy for nonrenal lupus erythematosus. (1980) (54)
- Cathepsin B Generates the Most Common Form of Amyloid A (76 Residues) as a Degradation Product from Serum Amyloid A (1995) (53)
- Tertiary structures of amyloidogenic and non-amyloidogenic transthyretin variants: new model for amyloid fibril formation. (1998) (52)
- Hereditary systemic amyloidosis associated with a new apolipoprotein AII stop codon mutation Stop78Arg. (2003) (52)
- Differential plasma clearance of murine acute-phase serum amyloid A proteins SAA1 and SAA2. (1997) (51)
- Treatment of AL amyloidosis with melphalan, prednisone, and colchicine. (1986) (51)
- Suppression of in vitro antibody response by a serum factor (SAA) in experimentally induced amyloidosis (1975) (51)
- Nomenclature of amyloid and amyloidosis (1998) (50)
- Localization of human SAA gene(s) to chromosome 11 and detection of DNA polymorphisms. (1986) (49)
- In Vitro Degradation of Serum Amyloid A by Cathepsin D and Other Acid Proteases: Possible Protection Against Amyloid Fibril Formation (1995) (49)
- Senile cardiac amyloidosis associated with homozygosity for a transthyretin variant (ILE-122). (1991) (49)
- Renal amyloidosis caused by a novel stop-codon mutation in the apolipoprotein A-II gene. (2001) (47)
- The Ile-84-->Ser amino acid substitution in transthyretin interferes with the interaction with plasma retinol-binding protein. (1994) (47)
- Contribution of wild‐type transthyretin to hereditary peripheral nerve amyloid (2003) (47)
- Reduced affinity for thyroxine in two of three structural thyroxine-binding prealbumin variants associated with familial amyloidotic polyneuropathy. (1986) (46)
- Hereditary amyloidosis: detection of variant prealbumin genes by restriction enzyme analysis of amplified genomic DNA sequences (1990) (46)
- A new mutant transthyretin (Arg 10) associated with familial amyloid polyneuropathy. (1992) (45)
- Suppression of choroid plexus transthyretin levels by antisense oligonucleotide treatment (2010) (45)
- Biochemical characterisation of amyloid by endomyocardial biopsy (2009) (44)
- Treatment of Wegener's granulomatosis with immunosuppressive agents. Description of renal ultrastructure. (1970) (43)
- Identification of carriers of a variant plasma prealbumin (transthyretin) associated with familial amyloidotic polyneuropathy type I. (1985) (42)
- Accelerated amyloid deposition in mice treated with the aspartic protease inhibitor, pepstatin. (1996) (42)
- NEUROPATHY, M COMPONENTS, AND AMYLOID (1975) (41)
- Synovial fluid concentrations of diclofenac in patients with rheumatoid arthritis or osteoarthritis. (1985) (41)
- Inotersen therapy of transthyretin amyloid cardiomyopathy (2019) (41)
- "A" protein of amyloidosis. Isolation of a cross-reacting component from serum by affinity chromatography. (1975) (41)
- Biochemical and clinical characterization of prealbuminCHICAGO: an apparently benign variant of serum prealbumin (transthyretin) discovered with high-resolution two-dimensional electrophoresis. (1991) (40)
- The SJL/J mouse: a new model for spontaneous age-associated amyloidosis. I. Morphologic and immunochemical aspects. (1976) (40)
- P-component of amyloid. Isolation from human serum by affinity chromatography. (1976) (39)
- Polymorphism in a kappa I primary (AL) amyloid protein (BAN). (1986) (39)
- Induction of beta-sheet structure in amyloidogenic peptides by neutralization of aspartate: a model for amyloid nucleation. (1999) (38)
- Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis (2007) (38)
- Assessing mNIS+7Ionis and international neurologists' proficiency in a familial amyloidotic polyneuropathy trial (2017) (38)
- Pathogenesis of transthyretin amyloidosis (2012) (36)
- Serum amyloid a in carcinoma of the lung (1986) (36)
- Structural analyses of fibrinogen amyloid fibrils (2007) (36)
- Thyroxine interactions with transthyretin: a comparison of 10 different naturally occurring human transthyretin variants. (1993) (35)
- Isolation and characterization of amyloid protein AA in the Abyssinian cat. (1985) (35)
- Aging in heterozygous Dnmt1-deficient mice: effects on survival, the DNA methylation genes, and the development of amyloidosis. (2006) (35)
- Scalloped pupils in familial amyloidosis. (1975) (34)
- Early data on long‐term efficacy and safety of inotersen in patients with hereditary transthyretin amyloidosis: a 2‐year update from the open‐label extension of the NEURO‐TTR trial (2020) (34)
- Serum amyloid P-component levels in amyloidosis, connective tissue diseases, infection, and malignancy as compared to normal serum. (1979) (34)
- Amyloid Fibril Protein Related to Immunoglobulin λ-Chains (1975) (34)
- Amyloid deposition in a renal transplant in familial Mediterranean fever. (1977) (34)
- A DNA test for Indiana/Swiss hereditary amyloidosis (FAP II). (1988) (33)
- Rate of progression of transthyretin amyloidosis. (2011) (33)
- Antisense oligonucleotide therapy for TTR amyloidosis (2011) (33)
- MOLECULAR GENETICS OF AMYLOID NEUROPATHY IN EUROPE (1989) (33)
- Biochemical characterization of vitreous and cardiac amyloid in Ile84Ser transthyretin amyloidosis (2006) (33)
- Antinuclear antibodies in systemic lupus erythematosus. Detection with horseradish-peroxidase-conjugated antibody. (1970) (33)
- Clinical characteristics in a kindred with early‐onset Alzheimer's disease and their linkage to a G→T change at position 2149 of the amyloid precursor protein gene (1994) (33)
- Genetic microheterogeneity of human transthyretin detected by IEF (2007) (32)
- Biochemical characterization of a neuroserpin variant associated with hereditary dementia. (2001) (32)
- Low plasma concentrations of retinol-binding protein in individuals with mutations affecting position 84 of the transthyretin molecule. (1995) (32)
- A trinucleotide deletion in the transthyretin gene (AV122) in a kindred with familial amyloidotic polyneuropathy (1997) (32)
- Spectrum of amyloid β-protein immunoreactivity in hereditary Alzheimer disease with a guanine to thymine missence change at position 1924 of the APP gene (1992) (31)
- Biophysical analysis of normal transthyretin: Implications for fibril formation in senile systemic amyloidosis (2001) (31)
- A transthyretin variant (alanine 71) associated with familial amyloidotic polyneuropathy in a French family. (1993) (30)
- Biochemical characteristics of variant transthyretins causing hereditary leptomeningeal amyloidosis (2005) (30)
- Amyloidosis of the shoulder in patients on chronic hemodialysis: sonographic findings. (1996) (29)
- Production and functional analysis of normal and variant recombinant human transthyretin proteins. (1992) (29)
- Renal transplantation for apolipoprotein AII amyloidosis (2003) (29)
- Inherited amyloidosis. (1991) (29)
- Ligand conjugated antisense oligonucleotide for the treatment of transthyretin amyloidosis: preclinical and phase 1 data (2020) (29)
- Amino acid sequence of a kappa I primary (AL) amyloid protein (AND). (1990) (28)
- Alteration in molecular structure which results in disease: the Met-30 variant of human plasma transthyretin. (1992) (28)
- Identification of a novel substitution in the constant region of a gene coding for an amyloidogenic kappa1 light chain. (1999) (28)
- Cellular events associated with the initial phase of AA amyloidogenesis: insights from a human monocyte model (2007) (27)
- Late‐onset familial amyloid polyneuropathy in an American family of English origin (1989) (27)
- Diffuse fasciitis and eosinophilia with symmetric polyarthritis. (1980) (26)
- Amino Acid Sequence of a λ VI Primary (AL) Amyloid Protein (WLT) (1985) (26)
- Antisense oligonucleotide suppression of serum amyloid A reduces amyloid deposition in mice with AA amyloidosis (2011) (24)
- A novel variant of transthyretin (Glu89Lys) associated with familial amyloidotic polyneuropathy (2000) (24)
- The molecular basis of renal amyloidosis in Irish-American and Polish-Canadian kindreds. (1996) (24)
- A transthyretin mutation (Tyr78Phe) associated with peripheral neuropathy, carpal tunnel syndrome and skin amyloidosis (2003) (24)
- Local synthesis of amyloid fibril precursor in AL amyloidosis of the urinary tract. (1998) (23)
- Inflammation-Responsive Transcription Factor SAF-1 Activity Is Linked to the Development of Amyloid A Amyloidosis (2006) (23)
- Amyloid-enhancing factor mediates amyloid formation on fibroblasts via a nidus/template mechanism. (2003) (23)
- Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American-Finnish families (2004) (22)
- Primary structures of dog and cat amyloid A proteins: comparison to human AA. (1989) (22)
- Metabolism of amyloid proteins. (1996) (22)
- Identification and characterization of amyloid protein AA in spontaneous canine amyloidosis. (1985) (21)
- Measurement of serum amyloid a4 (SAA4): Its constitutive presence in serum (1994) (21)
- Azathioprine therapy in polymyositis. (1973) (21)
- Guidelines and new directions in the therapy and monitoring of ATTRv amyloidosis (2022) (21)
- A pair of peptides inhibits seeding of the hormone transporter transthyretin into amyloid fibrils (2019) (21)
- Inotersen (transthyretin-specific antisense oligonucleotide) for treatment of transthyretin amyloidosis. (2019) (21)
- Thermodynamic stability of a kappaI immunoglobulin light chain: relevance to multiple myeloma. (2005) (21)
- Amyloid polyneuropathy in two German-American families: a new transthyretin variant (Val 107). (1994) (20)
- Transthyretin amyloidosis (serine 44) with headache, hearing loss, and peripheral neuropathy (1998) (19)
- Azathioprine Therapy in Polymyositis (1973) (19)
- Haplotype analysis of common transthyretin mutations (1995) (19)
- Sarcoidosis and immunoglobulin lambda II light-chain amyloidosis diagnosed after orthotopic heart transplantation: a case report and review of the literature (2005) (18)
- A new transthyretin variant (His 69) associated with vitreous amyloid in an FAP family (1994) (18)
- Hereditary systemic immunoglobulin light-chain amyloidosis. (2015) (18)
- Transthyretin mutation (serine 84) associated with familial amyloid polyneuropathy in a Hungarian family. (1998) (18)
- Cardiac amyloidosis associated with the transthyretin Ile122 mutation in a Caucasian family (2001) (18)
- Uncertain value of urinary sediments in the diagnosis of amyloidosis. (1977) (18)
- Fractionation of primary amyloid fibrils. Characterization and chemical interaction of the subunits. (1977) (18)
- A new transthyretin variant (Ser 24) associated with familial amyloid polyneuropathy. (1995) (18)
- Diagnosis of familial amyloidotic polyneuropathy in France (1990) (17)
- Comparison of the Amino Acid Sequences of Ten Kappa I Amyloid Proteins for Amyloidogenic Sequences (1991) (17)
- Asp187Asn mutation of gelsolin in an American kindred with familial amyloidosis, Finnish type (FAP IV) (1995) (17)
- Fibrinogen A alpha chain Leu 554: an African-American kindred with late onset renal amyloidosis. (1998) (17)
- Threonine for alanine substitution at position 109 of transthyretin differentially alters human transthyretin's affinity for iodothyronines. (1994) (17)
- Familial and Senile Amyloidosis Caused by Transthyretin (2010) (17)
- Inotersen preserves or improves quality of life in hereditary transthyretin amyloidosis (2019) (17)
- Hereditary transthyretin amyloidosis: baseline characteristics of patients in the NEURO-TTR trial (2018) (17)
- Transthyretin isoleucine-122 mutation in African and American blacks (2000) (17)
- Structural changes in transthyretin produced by the Ile 84 Ser mutation which result in decreased affinity for retinol-binding protein (1996) (16)
- Laboratory Assessment of Transthyretin Amyloidosis (2002) (16)
- Tissue biopsy for the diagnosis of amyloidosis: experience from some centres (2021) (16)
- Shoulder‐Pad Sign of Amyloidosis: Structure of an Ig Kappa III Protein (2001) (16)
- LECT2 amyloidosis. (2010) (16)
- Molecular detection of carriers of hereditary amyloidosis in a Swedish-American family. (1986) (15)
- Relationship of migraine headache and stroke to oral contraceptive use. (1986) (15)
- Primary structure of amyloid fibril protein AA in azocasein-induced amyloidosis of CBA/J mice. (1987) (15)
- Prenatal detection of a gene for hereditary amyloidosis. (1989) (15)
- Hereditary amyloidosis: evidence against early amyloid deposition. (1989) (15)
- Hereditary amyloidosis and cardiomyopathy. (1992) (15)
- A transthyretin variant (alanine 49) associated with familial amyloidotic polyneuropathy in a French family. (1993) (15)
- Spinal claudication in systemic amyloidosis. (1989) (15)
- Diagnosis of Maryland/German familial amyloidotic polyneuropathy using allele‐specific, enzymatically amplified, genomic DNA (1990) (15)
- Amyloidoma of a spinal root (2003) (14)
- Familial amyloid polyneuropathy: Alanine‐for‐threonine substitution in the transthyretin (prealbumin) molecule (1990) (14)
- Rapidly progressive amyloid polyneuropathy associated with a novel variant transthyretin serine 25 (2002) (14)
- The primary structure of serum amyloid A protein in the rabbit: comparison with serum amyloid A proteins in other species. (1991) (14)
- Analysis of cDNA sequences of feline SAAs (2004) (14)
- Design and Rationale of the Global Phase 3 NEURO-TTRansform Study of Antisense Oligonucleotide AKCEA-TTR-LRx (ION-682884-CS3) in Hereditary Transthyretin-Mediated Amyloid Polyneuropathy (2021) (14)
- A rare transthyretin mutation (Asp18Glu) associated with cardiomyopathy (2004) (14)
- SAA SUPPRESSION OF IN VITRO ANTIBODY RESPONSE (1982) (14)
- Amyloidosis Related to a λ IV Immunoglobulin Light Chain Protein (1989) (13)
- Aging, amyloid, and cardiomyopathy. (1997) (13)
- Effects of Voltaren on arachidonic acid metabolism in arthritis patients. (1985) (13)
- Transthyretin amyloidosis associated with a novel variant (Trp41Leu) presenting with vitreous opacities (2002) (13)
- Chemical classification of hereditary amyloidosis in Brazilian families and identification of gene carriers. (1986) (13)
- Characterization of an isoelectric focusing variant of SAA1 (ASP-72) in a family of Turkish origin. (1991) (13)
- Liver Transplantation: An Effective Treatment For Familial ATTR Amyloidosis (2002) (13)
- Antigenic Determinants in Amyloid Deposits (1970) (13)
- Antigenicity and cross-reactivity of denatured fibril proteins of primary, secondary, and myeloma associated amyloids. (1975) (12)
- Treatment of transthyretin cardiomyopathy with a TTR-specific antisense oligonucleotide (IONIS-TTRRx) (2017) (12)
- Identity of a peritoneal fluid immunoglobulin light chain and the amyloid fibril in primary amyloidosis. (1976) (12)
- Diagnosis and Screening of Patients with Hereditary Transthyretin Amyloidosis (hATTR): Current Strategies and Guidelines (2020) (12)
- A new transthyretin mutation associated with leptomeningeal amyloidosis (2011) (12)
- A monoclonal immunoglobulin A (kappa) factor VIII:C inhibitor associated with primary amyloidosis: identification and characterization. (1989) (12)
- Familial oculoleptomeningeal amyloidosis associated with primary angiitis of the CNS (2007) (11)
- Chemical characterization of a lambda I amyloid protein isolated from formalin-fixed and paraffin-embedded tissue sections (2004) (11)
- Tracheobronchial amyloidosis: A review of clinical and radiographic characteristics, bronchoscopic diagnosis, and management (1998) (11)
- In vivo protein metabolism utilizing stable isotopes and mass spectrometry: a new approach to the study of mutant proteins in humans. (1990) (11)
- A cell-based high-throughput screening method to directly examine transthyretin amyloid fibril formation at neutral pH (2019) (11)
- Development of lines of transgenic mice expressing the human transthyretin Ser84 variant (1996) (11)
- Genetics: Clinical Implications of Transthyretin Amyloidosis (2009) (11)
- IN VITRO SYNTHESIS OF THE ACUTE PHASE REACTANT SAA BY HEPATOCYTES (1982) (11)
- A new transthyretin variant Leu55Gln in a patient with systemic amyloidosis (2002) (10)
- Kappa III immunoglobulin light chain origin of localized orbital amyloidosis (2004) (10)
- Erosive arthritis in hereditary amyloidosis. (1983) (10)
- The pathologic spectrum of oculoleptomeningeal amyloidosis with Val30Gly transthyretin gene mutation in a postmortem case. (2014) (10)
- Carbamylation of the amino‐terminal residue (Gly1) of mouse serum amyloid A promotes amyloid formation in a cell culture model (2016) (10)
- A European family with histidine 58 transthyretin mutation in familial amyloid polyneuropathy (1997) (10)
- Haplotype analysis of the transthyretin gene: Evidence for multiple recurrence of the Met30 mutation in the Caucasian population (1995) (10)
- Genetic amyloidosis: recent advances. (1989) (10)
- Hereditary amyloidosis--disease entity and clinical model. (1988) (9)
- Ovarian tumor antigens. (1983) (9)
- Solid organ transplantation for non-TTR hereditary amyloidosis: report from the 1st International Workshop on the Hereditary Renal Amyloidoses (2012) (9)
- Preparation and crystallization of human transthyretin (prealbumin) variants. (1991) (9)
- Characterization of Amyloid Familial β-Peptide in Familial Alzheimer′s Disease with APP717 Mutations (1993) (9)
- Treatment of ATTR cardiomyopathy with a TTR specific antisense oligonucleotide, inotersen (2019) (8)
- Prenatal diagnosis of hereditary amyloidosis in a Portuguese family. (1991) (8)
- Identification of a New Transthyretin Variant (Ile49) in Familial Amyloidotic Polyneuropathy Using Electrospray Ionization Mass Spectrometry and Nonisotopic RNase Cleavage Assay (1999) (8)
- Plasmacytoma of the nasal cavity with lambda III amyloid deposition (1995) (8)
- Lack of association of a restriction fragment length polymorphism for serum amyloid P gene with reactive amyloidosis. (1989) (8)
- Amyloidoma of the Esophagus (2007) (8)
- Amyloidosis: diagnosis and new therapies for a misunderstood and misdiagnosed disease. (2019) (8)
- Metabolism of normal and Met30 transthyretin. (1996) (8)
- IMPROVED SURVIVAL OF PATIENTS WITH TRANSTHYRETIN AMYLOID CARDIOMYOPATHY WITH INOTERSEN (TTR SPECIFIC ANTISENSE OLIGONUCLEOTIDE) (2019) (7)
- AA amyloidosis: potential therapy with antisense oligonucleotides (2011) (7)
- DNA sequence evidence for polymorphic forms of human serum amyloid A (SAA) (1986) (7)
- Rationale and Design of a Phase 3 Study to Evaluate the Efficacy and Safety of ION-682884 in Patients with Transthyretin-Mediated Amyloid Cardiomyopathy (ATTR-CM) (2019) (7)
- Isolated vitreoretinal amyloidosis in the absence of transthyretin mutations. (2003) (7)
- A 25,000 molecular weight protein constituent of human amyloid fibrils related to amyloid protein AA. (1975) (7)
- Recombinant murine serum amyloid A from baculovirus-infected insect cells: purification and characterization. (1993) (7)
- Haplotype analysis of His 58, Ala 60 and Tyr 77 types of familial amyloidotic polyneuropathy (1994) (6)
- Characterization of amyloid fibril beta-peptide in familial Alzheimer's disease with APP717 mutations. (1993) (6)
- Amino terminal sequence of amyloid P-component isolated from serum. (1978) (6)
- Fibrinogen A alpha-chain amyloidosis: report of the first case in Latin America (2013) (6)
- Report and recommendations from the 1st International Workshop on the Hereditary Renal Amyloidoses, FAP Symposium, London, 2008 (2010) (6)
- A transgenic mouse model of human systemic ApoA2 amyloidosis (2011) (6)
- Apolipoprotein AI and amyloidosis: a genetic model for aging. (1998) (6)
- Amino acid sequence of a lambda VI primary (AL) amyloid protein (WLT). (1985) (6)
- RFLP analysis for APP 717 mutations associated with Alzheimer's disease. (1993) (6)
- Human SAA1-derived amyloid deposition in cell culture: a consistent model utilizing human peripheral blood mononuclear cells and serum-free medium (2013) (6)
- Identification of a novel non-amyloidogenic transthyretin polymorphism (His 74) in the German population (1994) (6)
- Acute-phase reactants. (1989) (6)
- Expression of SAA and amyloidogenesis in congenic mice of CE/J and C57BL/6 strains (2000) (5)
- POTENTIAL REVERSAL OF TRANSTHYRETIN AMYLOID CARDIOMYOPATHY WITH TTR SPECIFIC ANTISENSE OLIGONUCLEOTIDE THERAPY (2018) (5)
- Abyssinian Cat Model of AA Amyloidosis: SAA Gene Analysis (1991) (5)
- Hairy cell leukemia and light chain (AL) amyloidosis: a case report (2011) (5)
- Amyloidosis of the Gastrointestinal Tract and Liver (2012) (5)
- Systemic AL Amyloidosis in a Cat (1991) (5)
- Treatment of transthyretin (TTR) amyloid cardiomyopathy with an antisense oligonucleotide inhibitor of TTR synthesis (2015) (5)
- Systemic immunoglobulin (AL) amyloidosis in a cat: complete primary structure of a feline lambda light chain (1996) (4)
- Lambda II immunoglobulin light chain protein in primary localized rectal amyloidosis (2007) (4)
- Amyloid fibril protein related to immunoglobulin lambda-chains. (1975) (4)
- Codeposition of transthyretin and immunoglobulin lambda light chain in senile cardiac (ATTR) amyloidosis (2004) (4)
- Liver transplant alone without kidney transplant for fibrinogen Aα-chain (AFib) renal amyloidosis (2016) (4)
- κ I light chain AL amyloidosis presenting with rapidly progressive renal and hepatic failure with unusual renal amyloid distribution. (2012) (4)
- Transthyretin amyloidosis: a little history of hereditary amyloidosis (2017) (4)
- Biochemical characterization of vitreous amyloid formed after liver transplantation (2016) (4)
- Other Systemic Forms of Amyloidosis (2010) (4)
- Amyloidosis related to a lambda IV immunoglobulin light chain protein. (1989) (3)
- Cyclosporin Reduces the Incidence of Amyloid Disease in Casein Treated Mice (1991) (3)
- Rapidly progressive AA cardiomyopathy (2011) (3)
- A Phase 3 Study to Evaluate ISIS-TTRRx in Patients with Transthyretin Familial Amyloid Polyneuropathy (TTR-FAP): Study Design and Baseline Demographics (S50.006) (2015) (3)
- Chapter 88 – Peripheral Neuropathies (2015) (3)
- SAFETY OF INOTERSEN TREATMENT IN PATIENTS WITH TRANSTHYRETIN AMYLOID CARDIOMYOPATHY (2019) (3)
- INOTERSEN IMPROVES QUALITY OF LIFE IN PATIENTS WITH HEREDITARY TRANSTHYRETIN AMYLOIDOSIS WITH POLYNEUROPATHY AND CARDIOMYOPATHY: RESULTS OF THE PHASE 3 STUDY NEURO-TTR (2018) (3)
- Sequence Analysis of a Third Human SAA Gene (1991) (3)
- Amyloidosis and Other Protein Deposition Diseases (2013) (3)
- Disease‐free survival following high dose or standard dose therapy in patients with amyloidosis (2016) (3)
- A transgenic mouse model reproduces human hereditary systemic amyloidosis. (2019) (2)
- Long-Term Update from the Open-Label Extension of the NEURO-TTR Study in Patients with Hereditary Transthyretin Amyloidosis (2018) (2)
- Long-term efficacy and safety of inotersen for hereditary transthyretin amyloidosis: NEURO-TTR open-label extension 3-year update (2022) (2)
- In frame deletion in the transthyretin gene (ΔV122) associated with amyloidotic polyneuropathy (1996) (2)
- DNA Tests for Four Prealbumin Mutations in Hereditary Amyloidosis (1988) (2)
- Open Label Extension of the Phase 3 Study NEURO-TTR to Assess the Long-term Efficacy and Safety of Inotersen in Patients With Hereditary Transthyretin Amyloidosis (P1.324) (2018) (2)
- Exploring Mechanisms of Inhibition of Amyloid Seeding of Transthyretin (2018) (2)
- Inotersen treatment for ATTR amyloidosis (2019) (2)
- 3 year follow-up results on a treatment for amyloid A (AA) amyloidosis: Efficacy and safety of eprodisate (NC-503; 1,3-propanedisulfonate), the first of a new class of anti-amyloid compounds. (2006) (2)
- Pathofibrillogenesis and Amyloid Proteins (1991) (2)
- Identification of a new prealbumin variant tyr 77 associated with autosomal dominant amyloidosis (1986) (2)
- Light Chain Amyloid Organ-Specific ( Localized ) Synthesis of Ig (1999) (2)
- Predominance of One SAA Isotype (SAAI) in Human Reactive Amyloid (1991) (2)
- Differential Regulation of Human Serum Amyloid a Isoforms (1991) (2)
- Suppression of in vitro antibody response of human peripheral blood lymphocytes by a heat-labile factor in normal human serum. (1981) (2)
- Plasma retinol binding protein levels in individuals with transthyretin gene mutations (1996) (1)
- PS1221 LONG-TERM EFFICACY AND SAFETY OF INOTERSEN FOR HEREDITARY TRANSTHYRETIN AMYLOIDOSIS: NEURO-TTR OPEN-LABEL EXTENSION 2-YEAR UPDATE (2019) (1)
- Familial Amyloidotic Polyneuropathy Type I: Characterization of the Prealbumin Amyloid Subunit and Precursor Protein (1986) (1)
- Non-Transthyretin Hereditary Amyloidoses (2004) (1)
- An aggressive form of transthyretin amyloidosis (2017) (1)
- Methylprednisolo ne pulse therapy fornonrenal lupus erythematosus (1980) (1)
- Some Structural Factors Involved in Amyloid Fibril Formation by Lambda VI Light Chain Proteins (1986) (1)
- Prealbumin and retinol binding protein levels in heredofamilial amyloidosis (1982) (1)
- Myelin P2 Protein in Transthyretin (Ser-84) Vitreous Amyloid (1991) (1)
- X-Ray Crystal Structure of the Met-30 Variant of Human Prealbumin (Transthyretin) (1991) (1)
- Disulfide Bond Formation of Transthyretin (TTR) as Possible Initial Step of Familial Amyloid Polyneuropathy (FAP) (1991) (1)
- Post-translational modification of amyloid a protein in patients with AA amyloidosis (2021) (1)
- Tandem intermediate dose melphalan supported by autologous progenitor cells in the management of primary amyloidosis (2000) (1)
- Amyloidogenic property of murine serum amyloid A isotypes (1996) (1)
- Familial Amyloidotic Polyneuropathy Type I and Type II: Characterization of Two Distinct Genetic Defects and Identification of Carriers of Each Gene (1986) (1)
- Structural Analysis of a Lambda IV Primary Amyloid Protein (1988) (1)
- Structural polymorphism of amyloid fibrils in cardiac ATTR amyloidosis revealed by cryo-electron microscopy (2022) (1)
- Burden of Hereditary Transthyretin Amyloidosis With Polyneuropathy in Patients Enrolled in the Phase 3 Study NEURO-TTR (P1.331) (2018) (1)
- Insulin: a diagnostic warning (2017) (1)
- Continued deposition of wild-type transthyretin in cardiac amyloid after liver transplantation (2004) (1)
- Amyloid Cardiomyopathy. (2016) (1)
- Five kindreds with variant fibrinogen presenting renal amyloidosis (1996) (1)
- STRUCTURE AND FUNCTION OF SAA (1986) (1)
- Light chain (AL) amyloidosis in the central nervous system (CNS) (2011) (1)
- Suppression of Transthyretin Synthesis by Antisense Oligonucleotides (2004) (1)
- Tertiary structures of three amyloidogenic transthyretin variants and implications for amyloid fibril formation (1996) (0)
- Systemic Diseases: From Amyloidosis to Cryoglobulinemia Hereditary fibrinogen A (cid:1) -chain amyloidosis: Phenotypic characterization of a systemic disease and the role of liver transplantation. 2010 (2010) (0)
- Primary structure of an amyloid pr precursor in a heredofamilial polyn (amyloidosis/plasma protein/protein sequence/autosomal dominant) (2016) (0)
- Oculoleptomeningeal Amyloid Val30Gly TTR Mutation (2014) (0)
- Bortezomib (Velcade) treatment of AL amyloidosis: Indiana University experience (2011) (0)
- In Vivo Metabolism of a Mutant Apolipoprotein A-I (ARG-26) Associated with Hereditary Amyloidosis (1991) (0)
- 3 year follow-up results on a treatment for amyloid A (AA) amyloidosis (2006) (0)
- Thanks to Reviewers2003 (2003) (0)
- Long-term Treatment with Inotersen for Amyloid Transthyretin Amyloidosis Cardiomyopathy (2018) (0)
- Response observed during treatment for amyloid a (AA) amyloidosis with eprodisate (NC-503; 1,3-propanedisulfonate) after 2 years in patients with familial mediterranean fever in comparison to patients with rheumatoid arthritis. (2006) (0)
- L'amyloid-enhancing factor initie la formation d'amylose AA extracellulaire in vitro (2002) (0)
- Long-Term Efficacy and Safety of Inotersen for Hereditary Transthyretin Amyloidosis: NEURO-TTR Open-Label Extension 2-Year Update (S27.008) (2019) (0)
- Incidence of transthyretin Val122Ile amyloid mutation in African-Americans born in Indianapolis, Indiana, USA (2004) (0)
- TRANSTHYRETIN (DEL VAL122) (1998) (0)
- Predictors of survival among 60 patients with tissue proven primary cardiac amyloidosis (2013) (0)
- P684Inotersen improved quality of life, polyneuropathy and cardiomyopathy in a diverse group of patients with hereditary transthyretin amyloidosis in the phase 3 study NEURO-TTR (2018) (0)
- The future of amyloidosis (2004) (0)
- IMMUNOGLOBULIN LIGHT CHAIN PROTEIN (1995) (0)
- Phylogenetic Analysis of Amyloid a Protein (1988) (0)
- Structure, function and metabolism of transthyretin (1996) (0)
- Amyloid or asteroid? [1] (multiple letters) (2005) (0)
- A newmutanttransthyretin (Arg10)associated withfamilial amyloid polyneuropathy (1992) (0)
- Biochemical Characterization of Amyloid from a Rectal Amyloidoma (2007) (0)
- P685Hereditary transthyretin amyloidosis is associated with significant disease burden: analysis of the baseline characteristics of patients from the phase 3 study NEURO-TTR (2018) (0)
- Response: Hereditary fibrinogen A α-chain amyloidosis: phenotypic characterization and the role of liver transplantation (2010) (0)
- ALECT2 and NASH (2019) (0)
- Structure and Function of Recombinant Human Transthyretin (1991) (0)
- transthyretin variant (Ser 24) associated with familial amyloid polyneuropathy (0)
- 57. Hereditary amyloidosis related to mutations in fibrinogen A??-chain (1998) (0)
- Indication of a common origin of German and American Families with Familial Amyloidneuropathy Typ II (1999) (0)
- Beneficial Effects of Eprodisate (NC-503) for Patients with Amyloid A (AA) Amyloidosis: Results of a 2-Year, Multi-Center, Randomized, Placebo-Controlled Trial (2006) (0)
- Composition of Peripheral Nerve Amyloid after Orthotopic Liver Transplant for Fap (2007) (0)
- Aging-related cyclization of aspartate as the initial event in amyloid formation (2000) (0)
- Amyloid or Asteroid?—Reply (2005) (0)
- Clearance of variant transthyretin from serum after liver transplantation in patients with familial amyloidotic polyneuropathy (1996) (0)
- Structural comparison of amyloidogenic light-chain dimer in two crystal forms with non-amyloidogenic counterparts (1996) (0)
- P010. Evaluation of the Efficacy and Safety of AKCEA-TTR-LRx (ION-682884) in Patients with Transthyretin-mediated Amyloid Cardiomyopathy: The CARDIO-TTRansform Study (2021) (0)
- Antinuclear Antibodies in Systemic Lupus Erythematosus (2020) (0)
- Vitreous amyloidosis without a mutation in the transthyretin gene (1996) (0)
- Peripheral neuropathies (2020) (0)
- Amyloid fibrils with fragmented ATTR may be the rule in non-Val30Met ATTR amyloidosis (2011) (0)
- Suppression of Hepatic Transthyretin Synthesis by Antisense Oligonucleotides (2007) (0)
- Interferon Alfa Induce Tnf Elevations in vivo. Correlation with Other Acute Phase Reactants. (1991) (0)
- Amyloid production by human monocyte cultures (2004) (0)
- Systemic Diseases (2010) (0)
- IX International Symposium on Amyloidosis July 15–21, 2001 Budapest, Hungary (2002) (0)
- Serum Amyloid A: Characterization of Three cDNAS in One Individual and Complete Structure of their Corresponding Protein Products (1988) (0)
- A S52R mutation in neuroserpin is associated with myoclonic epilepsy and dementia (2000) (0)
- [Indications for simultaneous origin of a German and American family with type II hereditary amyloid neuropathy]. (1999) (0)
- Determination of the amino-terminal cleavage site of PRPRES in GSS F198S (2000) (0)
- Is the nucleotide 654 of the geisolin gene a mutational hot spot? (1996) (0)
- Amyloidosis causing gastric outlet obstruction in a patient with non-Hodgkin's lymphoma (2003) (0)
- Inotersen Improves Norfolk Quality of Life-Diabetic Neuropathy Measures in Patients With Hereditary Transthyretin Amyloidosis with Polyneuropathy in the Phase 3 Study NEURO-TTR (S5.006) (2018) (0)
- Safety and Efficacy of Inotersen in Patients With Hereditary Transthyretin Amyloidosis With Polyneuropathy (NEURO-TTR) (N2.001) (2018) (0)
- Abstract 9312: Predictors of Survival in Primary Cardiac Amyloidosis (2012) (0)
- Fibrinogen A alpha-chain amyloidosis: phenotypic characterisation and the role of liver transplantation (2010) (0)
- Subject Index Vol. 49, 1999 (1999) (0)
- Normal and variant transthyretin in a patient with familial amyloidotic polyneuropathy: DNA, mRNA, serum, and amyloid deposits (1996) (0)
- Emerging Therapies for Amyloidosis (2012) (0)
- Hereditary amyloidosis: Some words on the history and present status of our eminent subject (2000) (0)
- Editorial Commentary: Advances in transthyretin amyloid cardiomyopathy. (2020) (0)
- Reactive (AA) Amyloidosis in a 14 Year Old with No Predisposing Disease (1991) (0)
- AA cardiomyopathy (2017) (0)
- Biochemical characterization of an immunoglobulin lambda I light chain amyloid protein isolated from formalin-fixed paraffin-embedded tissue sections (2004) (0)
- NEUROPATHOLOGY AT THE CROSSROADS OF NEUROPSYCHIATRY AND GENETICS: NEW INSIGHTS INTO NEUROSERPIN ENCEPHALOPATHY (2003) (0)
- Non-TTR hereditary amyloidosis (2004) (0)
- Amyloid-determining residues in mouse serum amyloid A (2004) (0)
- Proceedings: Bence jones protein (BJP) from a patient with primary amyloidosis identical to the amyloid fibril. (1975) (0)
- 2 The hereditary amyloidoses (2004) (0)
- Attr (Thr59lys) Amyloidosis with Involvement of Heart, Peripheral and Autonomic Nervous System, Gut and Salivary Glands in An Egyptian Man, Previously Diagnosed as “Familial Amyloid Neuropathy of Portuguese Type” (2007) (0)
- Efficacy and safety with >3 years of inotersen treatment for the polyneuropathy of hereditary transthyretin amyloidosis (2021) (0)
- HEREDITARY FIBRINOGEN A A ALPHA-CHAIN AMYLOIDOSIS; PHENOTYPIC CHARACTERIZATION AND THE ROLE OF LIVER TRANSPLANTATION: 702 (2008) (0)
- Biochemical Characterization of Leptomeningeal Amyloid in a Val30gly Transthyretin Patient (2007) (0)
- "Primary systemic amyloidosis": The seeds of our present research (1993) (0)
- Structural Analyses Of Fibrinogen Aalpha-Chain Amyloid Fibrils (2007) (0)
- In vitro translation of human serum amyloid A (1984) (0)
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