Kurt Von Figura
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Kurt Von Figurabiology Degrees
Biology
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Molecular Biology
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Biochemistry
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Biology
Kurt Von Figura's Degrees
- PhD Biochemistry University of California, San Francisco
- Bachelors Chemistry Stanford University
Why Is Kurt Von Figura Influential?
(Suggest an Edit or Addition)Kurt Von Figura'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
- Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein (1998) (1815)
- Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice (2000) (863)
- Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cells. (1995) (427)
- Multiple Sulfatase Deficiency Is Caused by Mutations in the Gene Encoding the Human Cα-Formylglycine Generating Enzyme (2003) (348)
- Intracellular receptor sorting during endocytosis: Comparative immunoelectron microscopy of multiple receptors in rat liver (1984) (320)
- Complementation cloning identifies CDG-IIc, a new type of congenital disorders of glycosylation, as a GDP-fucose transporter deficiency (2001) (310)
- Molecular Basis for Multiple Sulfatase Deficiency and Mechanism for Formylglycine Generation of the Human Formylglycine-Generating Enzyme (2005) (177)
- Lysosomal acid phosphatase is transported to lysosomes via the cell surface. (1989) (175)
- Genetic heterogeneity and clinical variability in the Sanfilippo syndrome (types A, B, and C) (1981) (172)
- Targeting of a lysosomal membrane protein: a tyrosine‐containing endocytosis signal in the cytoplasmic tail of lysosomal acid phosphatase is necessary and sufficient for targeting to lysosomes. (1990) (163)
- The essential tyrosine of the internalization signal in lysosomal acid phosphatase is part of a β turn (1991) (149)
- Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice (2003) (149)
- Mr 46,000 mannose 6‐phosphate specific receptor: its role in targeting of lysosomal enzymes. (1987) (136)
- Mannose 6‐phosphate receptor dependent secretion of lysosomal enzymes. (1990) (103)
- The cytoplasmic tail of lysosomal acid phosphatase contains overlapping but distinct signals for basolateral sorting and rapid internalization in polarized MDCK cells. (1993) (102)
- Human lysosomal acid phosphatase is transported as a transmembrane protein to lysosomes in transfected baby hamster kidney cells. (1988) (96)
- Carbohydrate-deficient glycoprotein syndromes become congenital disorders of glycosylation: An updated nomenclature for CDG (1999) (93)
- The Sanfilippo B corrective factor: A N-acetyl-α-D-glucosaminidase (1972) (88)
- Targeted disruption of the M(r) 46,000 mannose 6‐phosphate receptor gene in mice results in misrouting of lysosomal proteins. (1993) (84)
- Efficacy of enzyme replacement therapy in α-mannosidosis mice: a preclinical animal study (2004) (80)
- Antibody to mannose 6‐phosphate specific receptor induces receptor deficiency in human fibroblasts. (1984) (78)
- Regulation of the mannose 6‐phosphate/IGF II receptor expression at the cell surface by mannose 6‐phosphate, insulin like growth factors and epidermal growth factor. (1989) (70)
- Sequential processing of lysosomal acid phosphatase by a cytoplasmic thiol proteinase and a lysosomal aspartyl proteinase. (1989) (69)
- Human α‐N‐Acetylglucosaminidase (1977) (68)
- Oligosaccharides in Lysosomal Enzymes (1981) (66)
- Molecular recognition and targeting of lysosomal proteins. (1991) (66)
- The internalization signal in the cytoplasmic tail of lysosomal acid phosphatase consists of the hexapeptide PGYRHV. (1992) (61)
- Isolation and Characterization of Phosphorylated Oligosaccharides from α‐N‐Acetylglucosaminidase that Are Recognized by Cell‐Surface Receptors (1979) (59)
- LAMP-2 deficient mice show depressed cardiac contractile function without significant changes in calcium handling (2006) (57)
- Molecular genetics of metachromatic leukodystrophy (1994) (57)
- Myeloperoxidase is synthesized as larger phosphorylated precursor. (1984) (55)
- Targeted Disruption of the Lysosomal α-Mannosidase Gene Results in Mice Resembling a Mild form of Human α-Mannosidosis (1999) (54)
- Internalization of blocking antibodies against mannose‐6‐phosphate specific receptors. (1985) (51)
- Multiple Deficiency of Mucopolysaccharide Sulfatases in Mucosulfatidosis (1979) (47)
- Molecular analysis of SUMF1 mutations: stability and residual activity of mutant formylglycine‐generating enzyme determine disease severity in multiple sulfatase deficiency (2008) (40)
- Identification of three internalization sequences in the cytoplasmic tail of the 46 kDa mannose 6-phosphate receptor. (1997) (39)
- Secretion of β-hexosaminidase by cultured human skin fibroblasts: Kinetics, effect of temperature, cell density, serum concentration and pH☆ (1978) (38)
- Expression of mannose 6‐phosphate receptors in chicken (1996) (34)
- Four novel mutant alleles of the arylsulfatase B gene in two patients with intermediate form of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) (1994) (33)
- Sanfilippo A disease in the fetus (1974) (32)
- Morquio syndrome: Clinical findings in 11 patients with MPS IVA and 2 patients with MPS IVB (2004) (31)
- Sanfilippo type D disease: Clinical findings in two patients with a new variant of mucopolysaccharidosis III (1979) (30)
- Dephosphorylation of the Mannose‐6 Phosphate Recognition Marker is Localized in Late Compartments of the Endocytic Route (1996) (27)
- I-cell disease-like phenotype in mice deficient in mannose 6-phosphate receptors (1998) (27)
- Rapid equilibrium between monomeric, dimeric and tetrameric forms of the 46‐kDa mannose 6‐phosphate receptor at 37°C (1990) (26)
- MANGANESE deficiency. (2022) (24)
- Decreased availability of GDP‐l‐fucose in a patient with LAD II with normal GDP‐d‐mannose dehydratase and FX protein activities (1999) (23)
- The Phosphorylation Pattern of Oligosaccharides in Secreted Procathepsin D Is Glycosylation Site-specific and Independent of the Expression of Mannose 6-Phosphate Receptors* (1997) (23)
- Synthesis of lysosomal α-mannosidase in normal and mannosidosis fibroblasts (1983) (21)
- Sanfilippo type C disease: Clinical findings in four patients with a new variant of mucopolysaccharidosis III (2004) (20)
- Identification of the putative mannose 6-phosphate receptor (MPR 46) protein in the invertebrate mollusc. (2002) (20)
- Eukaryotic formylglycine‐generating enzyme catalyses a monooxygenase type of reaction (2015) (19)
- Mannosidosis. Storage of Mannose-containing Material in Cultured Human Mannosidosis Cells and Metabolic Correction by Pig Kidney α-Mannosidase (1976) (18)
- MANNOSE 6-PHOSPHATE RECEPTOR PROTEINS FROM REPTILES AND AMPHIBIANS : EVIDENCE FOR THE PRESENCE OF MPR 300 AND MPR 46 (1997) (17)
- Morphological and biochemical findings in a case of mucopolysaccharidosis type III A (Sanfilippo's disease type A). (1975) (16)
- Inhibition of Pinocytosis by Cytochalasin B (1974) (16)
- Genesis of lysosomal enzyme deficiencies (1984) (15)
- Molecular genetics of metachromatic leukodystrophy (1991) (14)
- Immunological characterization of human acid phosphatase gene products (1985) (13)
- Cloning and Expression of Human Steroid-sulfatase (2001) (12)
- The early vertebrate Danio rerio Mr 46000 mannose-6-phosphate receptor: biochemical and functional characterisation (2006) (12)
- Linkage analysis in X-linked ichthyosis (steroid sulfatase deficiency) (1988) (11)
- Sanfilippo syndrome type C: assay for acetyl‐CoA: α‐glucosaminide N‐acetyltransferase in leukocytes for detection of homozygous and heterozygous individuals (1981) (10)
- LYSOSOMAL STORAGE DISORDERS CAUSED BY INSTABILITY OF THE MISSING ENZYMES (1984) (9)
- An 11-bp deletion in the arylsulfatase A gene of a patient with late infantile metachromatic leukodystrophy (1991) (9)
- Mutations in arylsulfatase A alleles causing metachromatic leukodystrophy (1991) (9)
- Studies on the chemistry of arterial wall, XVII. Metabolic characteristics of different types of chondroitin sulfate-dermatan sulfate hybrids in arterial tissue. (1975) (7)
- Synthesis and Processing of a-Galactosidase A in Human Fibroblasts (1987) (7)
- Advances in the molecular genetics of metachromatic leukodystrophy (1990) (7)
- Deficiency of mannose 6‐phosphate receptors and lysosomal storage: a morphometric analysis of hepatocytes of neonatal mice (2003) (7)
- Demonstration of D‐glucuronic acid as reducing terminal of intracellular heparan sulfates (1976) (6)
- NormalN-oligosaccharyltransferase activity in fibroblasts from patients with carbohydrate-deficient glycoprotein syndrome (1994) (6)
- Effect of temperature on endocytosis and degradation of sulphated proteoglycans by cultured skin fibroblasts (1982) (6)
- Acid Hydrolase Production, Release and Uptake by Cultured Fibroblasts (1977) (6)
- Mannose 6-phosphate/insulin-like growth factor II receptor and I-cell disease fibroblasts: increased synthesis and defective regulation of cell surface expression (1992) (5)
- Transport and Processing of Lysosomal Enzymes (1986) (4)
- The mucopolysaccharidoses: Biochemistry and clinical symptoms (1981) (4)
- Mucopolysaccharidose I-S (Morbus Scheie) (1979) (3)
- Carbohydrate mediated recognition of lysosomal enzymes by cell surface receptors (1983) (3)
- Origin and Dynamics of Lysosomes (1980) (3)
- Structure-function relationship for lysosomal enzymes. (2007) (3)
- Different Types of Chondroitin Sulfate-Dermatan Sulfate Hybrids in Arterial Tissue (1975) (2)
- Mr 46 , 000 Mannose 6-Phosphate Receptor (2001) (2)
- Late onset form of mucopolysaccharidosis type I: Clinical aspect and biochemical characterization of residual α-l-iduronidase activity (1981) (2)
- Sulfatase Deficiencies in Mucopolysaccharidoses (1975) (2)
- A New Variant of Galactosemia: Galactose-1-phosphate Uridylytransferase Sensitive to Product Inhibition by Glucose 1-phosphate (1980) (2)
- Glucose and Lipid Metabolism in the Human Arteriosclerotic Aorta (1974) (2)
- Multiple Sulfatase Deficiency (2009) (2)
- SUMMARY μ 1 A deficiency induces a profound increase in MPR 300 / IGF-II receptor internalization rate (2)
- Acid phosphatase deficiency leads to accumulation of osteopontin in the subosteoclastic resorption area (2001) (1)
- 3 Å Structure of Arylsulfatase from Pseudomonas aeruginosa Establishes the Catalytic Mechanism of Sulfate Ester Cleavage in the Sulfatase (2001) (1)
- Mutation (AG,,,) Results in a Severe (1992) (1)
- Glycosidases: Their role in degradation of glycoconjugates and pathogenesis of lysosomal storage disorders (1982) (1)
- Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling the phenotype of human alpha-mannosidosis (1999) (1)
- Defects in lysosomal enzyme modification for catalytic activity (2004) (1)
- Arylsulfatase from Pseudomonas aeruginosa (2001) (1)
- Metabolic Heterogeneity of Acidic Glycosaminoglycans in Bovine Arterial Tissue (1974) (1)
- Index rerum, vol. 9 (1972) (0)
- Inherited disorders caused by faulty protein modification in the secretory route (2002) (0)
- Signal-Mediated Targeting of Lysosomal Membrane Glycoproteins (1994) (0)
- Human MIR-receptor, repeat 11 (2001) (0)
- Metabolic Fatty Liver Disease (2009) (0)
- Clinical and molecular characterization of patients with multiple sulfatase deficiency (2006) (0)
- Crystal structure of an Arylsulfatase A mutant C69S soaked in synthetic substrate (2001) (0)
- Table of Contents (1995) (0)
- Inhibition of Lysosomal Enzyme Endocytosis by Carbohydrate and Lectins (1979) (0)
- Murine Microglia Antigen Processing in Primary Cultured Involvement of Cathepsin E in Exogenous REGULATION : ENZYME CATALYSIS (2002) (0)
- Recognition Marker in Lysosomal Enzymes: Presence of α-N-acetylglucosamine(1)phospho(6)mannose Diester Groups (1981) (0)
- Correction of Lysosomal Enzyme Deficiences by Enzyme Replacement (1981) (0)
- Comparative studies of lysosomal enzyme uptake by cultivated fibroblasts, hepatocytes and non parenchymal liver cells: 52 (1980) (0)
- A mouse model for congenital disorder of glycosylation ia (2005) (0)
- Pathogenesis and Genetics of Metachromatic Leukodystrophy (1996) (0)
- HUMAN ARYLSULFATASE A (1998) (0)
- Mannose 6-phosphate-specific receptor is a transmembrane protein with a C-terminal extension oriented towards the cytosol (2005) (0)
- Crystal structure of an Arylsulfatase A mutant P426L (2001) (0)
- The nineteen amino acid cytoplasmic tail of lysosomal acid phosphatase contains an endocytosis signal necessary and sufficient for targeting to lysosomes (1992) (0)
- Effects of Brefeldin A on the Endocytic Route REDISTRIBUTION OF MANNOSE 6-PHOSPHATE / INSULIN-LIKE GROWTH FACTOR I 1 RECEPTORS (2001) (0)
- Cathepsin K deficient mice have impaired bone resorption (1998) (0)
- Specificity of Pinocytosis of α-N-acetylglucosaminidase by Fibroblasts (1975) (0)
- Cells that co-express an enzyme that generates a sulfatase and a C-formyliglycine and its methods and uses (2004) (0)
- Molecular basis for MSD and catalytic mechanism of the human formylglycine generating enzyme (2005) (0)
- DEFECTIVE PROCESSING OF LYSOSOMAL ENZYMES (1986) (0)
- S14.1 Mannose-6-phosphate receptors and lysosomes (1993) (0)
- Mild hyperphenylalaninemia (2020) (0)
- Structure-function relationships in a -galactosidase A. Commentary (2007) (0)
- Cloning of a cDNA encoding the human cation-dependent mannose 6-phosphate-specific receptor ( lysosomal enzyme transport / transmembrane protein / chromosomal assignment ) (0)
- Biosynthesis andMaturation ofa-N-Acetylglucosaminidase inNormalandSanfilippo B-Fibroblasts (1984) (0)
- 33 PROTEINASE INHIBITOR CBZ-PHE-ALA-CHN2 (CBZ): A DRUG FOR TREATMENT OF PATIENTS WITH METACRROMATIC LEUCO-DYSTROPHY (MLD)? (1988) (0)
- EUROGLYCAN: A systematic approach towards the understanding, diagnosis and treatment of CDG, a novel group of inborn metabolic disorders caused by defects of glycosylation (2002) (0)
- Contents, Vol. 9, 1972 (1972) (0)
- 148 LYSOSOMAL GLYCOGEN STORAGE DISEASE WITHOUT DEFICIENCY OF ACID α-GLUCOSIDASE (1986) (0)
- C(alpha)-Formylglycine: a novel protein modification in pro- and eukaryotes (2002) (0)
- Action of Chondroitinases ABC and AC on Chondroitin 4-Sulfate-Oligosaccharides with Terminal N-Acetylgalactosaminitol Residues (1978) (0)
- Processing of the Phosphorylated Recognition Marker in Lysosomal Enzymes AND PARTIAL PURIFICATION OF A MICROSOMAL (u-N-ACETYLGLUCOSAMINYL (1981) (0)
- The two major familial Alzheimer's Disease gene products, presenilin 1 and amyloid precursor protein interact functionally (1998) (0)
- Mannose 6-phosphate and growth-factor induced redistribution of mannose 6-phosphate IGF-II receptors. (1988) (0)
- Molecular Genetics and Pathogenesis of Metachromatic Leukodystrophy (1997) (0)
- Table of Contents (1989) (0)
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