James P. Kushner
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American hematologist
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James P. Kushner's Degrees
- Doctorate Medicine Harvard University
Why Is James P. Kushner Influential?
(Suggest an Edit or Addition)According to Wikipedia, James P. Kushner is an American hematologist, currently Distinguished Professor Emeritus at University of Utah.
James P. Kushner'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
- Prevalence of hemochromatosis among 11,065 presumably healthy blood donors. (1988) (625)
- Evidence for cytokine-inducible nitric oxide synthesis from L-arginine in patients receiving interleukin-2 therapy. (1992) (509)
- Recommendations for the Diagnosis and Treatment of the Acute Porphyrias (2005) (491)
- Biosynthesis of heme in mammals. (2006) (414)
- Clinical and biochemical abnormalities in people heterozygous for hemochromatosis. (1996) (280)
- An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity. (1976) (263)
- Tissue distribution and clearance kinetics of non-transferrin-bound iron in the hypotransferrinemic mouse: a rodent model for hemochromatosis. (1987) (244)
- Oxidative stress, beta-cell apoptosis, and decreased insulin secretory capacity in mouse models of hemochromatosis. (2004) (237)
- Screening for hemochromatosis. (1993) (185)
- Hepcidin mediates transcriptional changes that modulate acute cytokine-induced inflammatory responses in mice. (2010) (185)
- Hereditary hemochromatosis. Analysis of laboratory expression of the disease by genotype in 18 pedigrees. (1982) (185)
- Hemochromatosis genes and other factors contributing to the pathogenesis of porphyria cutanea tarda. (2000) (174)
- Disease-related conditions in relatives of patients with hemochromatosis. (2000) (168)
- Homogeneous multiplex genotyping of hemochromatosis mutations with fluorescent hybridization probes. (1998) (159)
- Secondary iron overload. (2001) (156)
- High prevalence of abnormal glucose homeostasis secondary to decreased insulin secretion in individuals with hereditary haemochromatosis (2006) (150)
- The hepcidin-binding site on ferroportin is evolutionarily conserved. (2008) (149)
- Regulation of HeLa cell transferrin receptors. (1982) (145)
- Congenital erythropoietic porphyria due to a mutation in GATA1: the first trans-acting mutation causative for a human porphyria. (2007) (141)
- Internalization and subcellular localization of transferrin and transferrin receptors in HeLa cells. (1983) (138)
- Hypogonadism in hemochromatosis: reversal with iron depletion. (1984) (122)
- The role of iron in the pathogenesis of porphyria cutanea tarda. II. Inhibition of uroporphyrinogen decarboxylase. (1975) (120)
- Haplotype analysis of hemochromatosis: evaluation of different linkage-disequilibrium approaches and evolution of disease chromosomes. (1997) (118)
- A porphomethene inhibitor of uroporphyrinogen decarboxylase causes porphyria cutanea tarda (2007) (117)
- Crystal structure of human uroporphyrinogen decarboxylase (1998) (106)
- Heme regulation of HeLa cell transferrin receptor number. (1984) (98)
- Molecular cloning and nucleotide sequence of a complete human uroporphyrinogen decarboxylase cDNA. (1986) (91)
- Maintaining iron balance in women blood donors of childbearing age: summary of a workshop (2002) (89)
- Prevalence of heterozygotes for hemochromatosis in the white population of the United States. (1995) (85)
- Regulation of iron absorption in Hfe mutant mice. (2002) (84)
- Gene expression patterns that correlate with hepatitis C and early progression to fibrosis in liver transplant recipients. (2006) (82)
- Porphyria cutanea tarda in three generations of a single family. (1978) (80)
- The role of iron in the pathogenesis of porphyria cutanea tarda. An in vitro model. (1972) (79)
- Structural basis for tetrapyrrole coordination by uroporphyrinogen decarboxylase (2003) (78)
- Crystal Structure of the Oxygen-dependant Coproporphyrinogen Oxidase (Hem13p) of Saccharomyces cerevisiae* (2004) (76)
- Purification and characterization of bovine hepatic uroporphyrinogen decarboxylase. (1983) (75)
- Transferrin receptors of human fibroblasts. Analysis of receptor properties and regulation. (1982) (73)
- A method for detecting recent selection in the human genome from allele age estimates. (2003) (69)
- Hereditary hemochromatosis: contributions of genetic analyses. (1981) (62)
- Uroporphyrinogen decarboxylase: a splice site mutation causes the deletion of exon 6 in multiple families with porphyria cutanea tarda. (1990) (61)
- Clinical consequences of iron overload in hemochromatosis homozygotes. (2003) (61)
- A mouse model of familial porphyria cutanea tarda. (2001) (59)
- Preserving the national blood supply. (2001) (58)
- Mapping the locus for hereditary hemochromatosis: localization between HLA-B and HLA-A. (1986) (57)
- Identification of a novel mutation (C321X) in HJV. (2004) (56)
- Heterozygosity for HLA-linked hemochromatosis as a likely cause of the hepatic siderosis associated with sporadic porphyria cutanea tarda. (1985) (55)
- Idiopathic refractory sideroblastic anemia Incidence and risk factors for leukemic transformation (1979) (52)
- A point mutation in the coding region of uroporphyrinogen decarboxylase associated with familial porphyria cutanea tarda. (1989) (51)
- Analysis of uroporphyrinogen decarboxylase complementary DNAs in sporadic porphyria cutanea tarda. (1993) (51)
- Increased insulin secretory capacity but decreased insulin sensitivity after correction of iron overload by phlebotomy in hereditary haemochromatosis (2006) (50)
- Cardiomyopathy after widely separated courses of adriamycin exacerbated by actinomycin‐D and mithramycin (1975) (49)
- Iron absorption in hypotransferrinemic mice. (1991) (49)
- Screening for hemochromatosis: phenotype versus genotype. (1998) (48)
- HLA-linked hemochromatosis alleles in sporadic porphyria cutanea tarda. (1989) (48)
- Biosynthesis of porphyrin precursors. Purification and characterization of mammalian L-alanine:gamma,delta-dioxovaleric acid aminotransferase. (1980) (45)
- Clostridial species in the pathogenesis of necrotizing enterocolitis in patients with neutropenia (1983) (45)
- Blood lead concentrations in hereditary hemochromatosis. (1994) (44)
- Thyroid disease in hemochromatosis. Increased incidence in homozygous men. (1983) (41)
- Hepatic siderosis and porphyria cutanea tarda: relation of iron excess to the metabolic defect. (1977) (40)
- Transferrin receptor function in hereditary hemochromatosis. (1984) (40)
- Cytochrome P450 induction, uroporphyrinogen decarboxylase depression, porphyrin accumulation and excretion, and gender influence in a 3-week rat model of porphyria cutanea tarda. (1997) (39)
- Coincidental nontransfusional iron overload and thalassemia minor: association with HLA-linked hemochromatosis. (1981) (39)
- Increased Glucose Disposal and AMP-dependent Kinase Signaling in a Mouse Model of Hemochromatosis* (2007) (37)
- Allelic association under map error and recombinational heterogeneity: a tale of two sites. (1998) (37)
- Fulminant amebic meningoencephalitis due to Acanthamoeba (1981) (37)
- Iron overload in hereditary spherocytosis: Association with hla‐linked hemochromatosis (1982) (36)
- Down-regulation of hepcidin in porphyria cutanea tarda. (2008) (36)
- Characterization and crystallization of human uroporphyrinogen decarboxylase (1997) (36)
- Functional consequences of naturally occurring mutations in human uroporphyrinogen decarboxylase. (2001) (34)
- Transdermal estrogen replacement therapy in postmenopausal women previously treated for porphyria cutanea tarda. (2000) (32)
- Elevated Methionine-tRNA Synthetase Activity in Human Colon Cancer 1 (1976) (32)
- Toll-like receptors mediate induction of hepcidin in mice infected with Borrelia burgdorferi. (2009) (32)
- Regulation of the Distribution of Tissue Iron (1988) (32)
- Mutation analysis of transferrin-receptor 2 in patients with atypical hemochromatosis. (2002) (29)
- Uroporphyrinogen decarboxylase in Saccharomyces cerevisiae. HEM12 gene sequence and evidence for two conserved glycines essential for enzymatic activity. (1992) (29)
- Neonatal hemochromatosis. Genetic analysis of transferrin-receptor, H-apoferritin, and L-apoferritin loci and of the human leukocyte antigen class I region. (1990) (27)
- Physiology: Mining the genome for iron (2000) (27)
- Uroporphyrinogen decarboxylase. A method for measuring enzyme activity. (1982) (26)
- The enzymatic defect in porphyria cutanea tarda. (1982) (26)
- Zebrafish as a model for defining the functional impact of mammalian ferroportin mutations. (2007) (24)
- Monoblastic transformation in chronic myelogenous leukemia: Presentation with massive hepatic involvement (1981) (23)
- Fast track to the porphyrias (2005) (22)
- Laboratory diagnosis of the porphyrias. (1991) (22)
- Porphyria cutanea tarda associated with disinfectant misuse. (1975) (22)
- The morbidity of hemochromatosis among clinically unselected homozygotes: preliminary report. (1994) (21)
- Substrate shuttling between active sites of uroporphyrinogen decarboxylase is not required to generate coproporphyrinogen. (2009) (21)
- Congenital erythropoietic porphyria, diminished activity of uroporphyrinogen decarboxylase and dyserythropoiesis. (1982) (21)
- Mutational analysis of human uroporphyrinogen decarboxylase. (1996) (20)
- High-performance liquid chromatography of porphyrin esters. Identification of mixed esters generated in sample preparation. (1981) (19)
- Assignment of uroporphyrinogen decarboxylase (UROD) to the pter----p21 region of human chromosome 1. (1985) (19)
- Measurement of Uroporphyrinogen Decarboxylase Activity (1999) (19)
- Another link in the chain (2004) (18)
- Identification of a novel mutation in the L‐ferritin IRE leading to hereditary hyperferritinemia‐cataract syndrome (2005) (17)
- Identification of amino acid changes affecting yeast uroporphyrinogen decarboxylase activity by sequence analysis of hem12 mutant alleles. (1992) (16)
- Erratum: Recommendations for the diagnosis and treatment of the acute porphyrias (Annals of Internal Medicine (2005) 142 (439-450)) (2005) (15)
- Two novel uroporphyrinogen decarboxylase (URO-D) mutations causing hepatoerythropoietic porphyria (HEP). (2007) (15)
- Hereditary haemochromatosis. (1982) (15)
- Reduction of porphyrins to porphyrinogens with palladium on carbon. (2009) (14)
- CYP3A-inducing agents and the attenuation of uroporphyrin accumulation and excretion in a rat model of porphyria cutanea tarda. (2000) (14)
- Crystal structure of the oxygen-dependent coproporphyrinogen oxidase (Hem13p) of Saccharomyces cerevisiae (2004) (14)
- The Role of Iron in the Pathogenesis (1972) (12)
- Southern blood club symposium: an update on selected aspects of hemochromatosis. (1990) (11)
- Hereditary hemochromatosis. (2002) (11)
- Uroporphyria in the uroporphyrinogen decarboxylase‐deficient mouse: Interplay with siderosis and polychlorinated biphenyl exposure (2002) (11)
- Disorders of excess iron. (1991) (11)
- Uroporphyria in the Cyp1a2-/- mouse. (2011) (11)
- Anti-Obesity and Pro-Diabetic Effects of Hemochromatosis (2014) (10)
- Screening for hemochromatosis. (1995) (10)
- Biosynthesis of porphyrin precursors: Kinetic studies on mammalian l-alanine: γ,δ-Dioxovaleric acid aminotransferase (1980) (10)
- Biosynthesis of porphyrins and heme from gamma, delta-dioxovalerate by intact hepatocytes. (1981) (9)
- Comparison of stainable liver iron between symptomatic and asymptomatic hemochromatosis homozygotes and their homozygous relatives. (1991) (9)
- Increased frequency of HLA-A3 in subjects with sporadic porphyria cutanea tarda. (1988) (9)
- The HLA class I locus: analysis of RFLPs in hereditary hemochromatosis. (1989) (9)
- Biosynthesis of 5-aminolevulinic acid and heme from 4,5-dioxovalerate in the rat. (1983) (9)
- Steady-state levels of uroporphyrinogen decarboxylase mRNA in lymphoblastoid cell lines from patients with familial porphyria cutanea tarda and their relatives. (1988) (9)
- Mapping genes responsible for strain-specific iron phenotypes in murine chromosome substitution strains. (2007) (8)
- Hemochromatosis: The iron phenotype of hemochromatosis heterozygotes (2000) (8)
- Accelerated development of uroporphyria in mice heterozygous for a deletion at the uroporphyrinogen decarboxylase locus (2001) (8)
- Congenital Erythropoietic Porphyria, β-Thalassemia Intermedia and Thrombocytopenia Due to a GATA1 Mutation. (2005) (7)
- Rebuttal to Beutler (2003) (7)
- Acquired myeloperoxidase deficiency and recurrent infections in a patient with acute myelomonocytic leukemia (1979) (7)
- A method for determining δ-aminolevulinic acid synthase activity in homogenized cells and tissues. (2015) (6)
- Increased Incidence in Homozygous Men (2016) (6)
- Hemochromatosis: Hemochromatosis: a genetic definition (2000) (6)
- Coincidental hemochromatosis and viral hepatitis. (1991) (6)
- Attenuation of polychlorinated biphenyl induced uroporphyria by iron deprivation. (2005) (6)
- The enzymatic decarboxylation of the naturally occurring isomers of uroporphyrinogen by human erythrocytes. (1979) (5)
- Hemochromatosis: Estimate of the frequency of morbid complications of hemochromatosis (2000) (5)
- The effect of glyoxalase I on the metabolism of 4,5-dioxovaleric acid. (1985) (5)
- Mapping recombinant events with molecular markers in hemochromatosis pedigrees. (1994) (5)
- Cytologic diagnosis and monitoring of Hodgkin's disease in cerebrospinal fluid: A case report (1987) (5)
- Iron Chelation by Deferoxamine Induces Autophagy (2008) (5)
- CXCR 4 expression is associated with survival in familial chronic lymphocytic leukemia , but CD 38 expression (2002) (4)
- Prevalence of the 281 (Gly→Glu) mutation in hepatoerythropoietic porphyria and porphyria cutanea tarda (2004) (4)
- Phase I trial of low-dose oral Clofarabine in myelodysplastic syndromes patients who have failed frontline therapy. (2015) (4)
- The Heme Biosynthesis Pathway and Clinical Manifestations of Abnormal Function (1999) (4)
- Sporadic Porphyria Cutanea Tarda in Individuals with HLA‐linked Hemochromatosis Allele(s) (1988) (4)
- Screening for hemochromatosis in healthy blood donors. Preliminary results. (1988) (4)
- Recombinations defining centromeric and telomeric borders for the hereditary haemochromatosis locus. (1997) (4)
- Retraction notice to: The hepcidin-binding site on ferroportin is evolutionarily conserved. (2014) (3)
- Screening for Hemochromatosis in Healthy Blood Donors (1988) (3)
- Biosynthesis of Porphyrin Precursors PURIFICATION AND CHARACTERIZATION OF MAMMALIAN L-ALANINE : + DIOXOVALERIC ACID AMINOTRANSFERASE * (2001) (3)
- Reconstitution of normal hepcidin expression in Hfe-deficient mice after liver transplantation: a new role of HFE in Kupffer cells? (2010) (2)
- The enzymatic defects in porphyria cutanea tarda and variegate porphyria. (1982) (2)
- Cellular Distribution of Porphyrins In Porphyria Cutanea Tarda (2010) (1)
- The Uroporphomethene Inhibitor Causitive for Porphyria Cutanea Tarda (PCT) Is Generated by Oxidation of Hydroxymethylbilane (HMB) (2008) (1)
- 2 out of 3 isn't bad. (2010) (1)
- Uroporphyrinogen Decarboxylase with mutation D86N (2003) (1)
- Thermostable erythrocyte rosette-forming lymphocytes in hereditary hemochromatosis. I. Identification in peripheral blood (1984) (1)
- Longitudinal study of a mouse model of familial porphyria cutanea tarda. (2009) (1)
- Familial porphyria cutanea tarda: hybridization analysis of the uroporphyrinogen decarboxylase locus. (1988) (1)
- A Phase I Trial of Low-Dose Protracted Oral Clofarabine for the Treatment of Myelodysplastic Syndromes Patients Who Have Failed Frontline Therapy (2010) (1)
- Structural and kinetic characterization of mutant human uroporphyrinogen decarboxylases. (2009) (1)
- Hepcidin mediates transcriptional changes that modulate acute cytokine-induced inflammatory responses in mice (Journal of Clinical Investigation (2010) 120, 7(2395-2405) DOI: 10.1172/JCI42011) (2012) (1)
- Hyper- and hypo-induction of cytochrome P450 activities with Aroclor 1254 and 3-methylcholanthrene in Cyp1a2(-/-) mice. (2009) (0)
- Biosynthesis of porphyrin precursors: kinetic studies on mammalian L-alanine: gamma,delta-dioxovaleric acid aminotransferase. (1980) (0)
- Single-chain UROD F217Y (YF) mutation (2009) (0)
- Gly156Asp mutant of Human UroD, human uroporphyrinogen III decarboxylase (2001) (0)
- Uroporphyrinogen Decarboxylase single mutant D86E in complex with coproporphyrinogen-I (2003) (0)
- UROD single-chain dimer (2009) (0)
- 453 A MOUSE MODEL OF HEMOCHROMATOSIS EXHIBITS DECREASED INSULIN SECRETORY CAPACITY COMPENSATED BY INCREASED INSULIN SENSITIVITY, AND RESISTANCE TO DIET-INDUCED OBESITY (2005) (0)
- Subject Index Vol. 50, 1989 (1989) (0)
- Uroporphyrinogen Decarboxylase G168R single mutant apo-enzyme (2007) (0)
- An Inhibitor of Uroporphyrinogen Decarboxylase (URO-D) Causes Porphyria Cutanea Tarda (PCT). (2006) (0)
- De Domenico I, Vaughn MB, Yoon D, Kushner JP, Ward DM, Kaplan J. Zebrafish as a model for defining the functional impact of mammalian ferroportin mutations. Blood. 2007;110(10):3780–3783. (2012) (0)
- Erratum: (The Journal of Clinical Investigation (March 1992) 89 (868)) (1992) (0)
- Introduction: hereditary disorders of iron metabolism. (2002) (0)
- Hepcidin-Independent Regulation of Dietary Iron Absorption (2008) (0)
- Contents, Vol. 50, 1989 (1989) (0)
- 116 IRON OVERLOAD CAUSES INCREASED INSULIN SENSITIVITY AND GLUCOSE DISPOSAL ACCOMPANIED BY INCREASED HEPATIC GLUCOSE RECYCLING FROM LACTATE. (2007) (0)
- Crystal Structure of Coproporphyrinogen Oxidase (CPO). (2004) (0)
- Single-chain UROD Y164G (GY) mutation (2009) (0)
- Maxwell Myer Wintrobe: Influential Teacher in the Field of Hematology (2007) (0)
- Uroporphyrinogen Decarboxylase in complex with coproporphyrinogen-III (2003) (0)
- HAMP and HVJ as Candidate Modifier Genes in Type1 Hereditary Hemochromatosis with High Iron Burden and in Porphyria Cutanea Tarda (PCT). (2006) (0)
- Uroporphyrinogen Decarboxylase G168R single mutant enzyme in complex with coproporphyrinogen-III (2007) (0)
- Abstract LB-178: Phase I trial of low-dose oral clofarabine in myelodysplastic syndromes patients who have failed frontline therapy. (2013) (0)
- Ile260Thr mutant of Human UroD, human uroporphyrinogen III decarboxylase (2001) (0)
- YEAST OXYGEN-DEPENDENT COPROPORPHYRINOGEN OXIDASE (2004) (0)
- human UROD mutant K297N (2009) (0)
- Mechanisms Regulating Iron Absorption over a Wide Range of Dietary Iron Content in the Mouse. (2005) (0)
- Biosynthesis of porphyrins and heme from y , & dioxovalerate by intact hepatocytes (0)
- Identification of amino acid changes affecting yeast uroporphyrinogen decarboxylase activity by sequence analysis of heml 2 mutant alleles (2005) (0)
- Uroporphyrinogen Decarboxylase Y164F mutant in complex with coproporphyrinogen-III (2003) (0)
- Hepatic ABC Transporter Expression in A Hemochromatosis‐Linked Murine Model of Porphyria Cutanea Tarda (2008) (0)
- RECYCLING OF RETICULOENDOTHELIAL IRON IN VIVO IN THE ABSENCE OF APOTRANSFERRIN (1987) (0)
- Uroporphyrinogen Decarboxylase single mutant D86G in complex with coproporphyrinogen-III (2003) (0)
- 86 HAMP AND HVJ AS CANDIDATE MODIFIER GENES IN TYPE 1 HEREDITARY HEMOCHROMATOSIS WITH HIGH IRON BURDEN AND IN PORPHYRIA CUTANEA TARDA. (2007) (0)
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