Kurt Von Figura
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Biology
Kurt Von Figura's Degrees
- PhD Molecular Biology University of California, San Francisco
- Bachelors Biochemistry University of California, Berkeley
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(Suggest an Edit or Addition)Kurt Von Figura's Published Works
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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
- Impaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient mice. (1998) (903)
- Lysosomal enzymes and their receptors. (1986) (794)
- The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts. (2002) (710)
- μ1A‐adaptin‐deficient mice: lethality, loss of AP‐1 binding and rerouting of mannose 6‐phosphate receptors (2000) (428)
- Carbohydrate-deficient glycoprotein syndrome type Ib. Phosphomannose isomerase deficiency and mannose therapy. (1998) (370)
- Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease). (2005) (344)
- Role of LAMP-2 in lysosome biogenesis and autophagy. (2002) (320)
- Cathepsin L deficiency as molecular defect of furless: hyperproliferation of keratinocytes and pertubation of hair follicle cycling (2000) (314)
- Crystal structure of human arylsulfatase A: the aldehyde function and the metal ion at the active site suggest a novel mechanism for sulfate ester hydrolysis. (1998) (299)
- A di‐leucine‐based motif in the cytoplasmic tail of LIMP‐II and tyrosinase mediates selective binding of AP‐3 (1998) (290)
- Phosphorylation of the AP2 μ subunit by AAK1 mediates high affinity binding to membrane protein sorting signals (2002) (270)
- Molecular basis of different forms of metachromatic leukodystrophy. (1991) (258)
- Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts. (2004) (244)
- Enzymatic phosphorylation of lysosomal enzymes in the presence of UDP-N-acetylglucosamine. Absence of the activity in I-cell fibroblasts. (1981) (241)
- Possible pathways for lysosomal enzyme delivery (1985) (237)
- Guanidinoacetate methyltransferase deficiency: the first inborn error of creatine metabolism in man. (1996) (222)
- Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy. (1996) (216)
- Thyroid functions of mouse cathepsins B, K, and L. (2003) (214)
- Biosynthesis and transport of cathepsin D in cultured human fibroblasts (1983) (214)
- Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons. (2000) (207)
- The Two Mannose 6-Phosphate Receptors Transport Distinct Complements of Lysosomal Proteins (*) (1995) (206)
- Arylsulfatase A pseudodeficiency: loss of a polyadenylylation signal and N-glycosylation site. (1989) (199)
- Enzymic diagnosis of the genetic mucopolysaccharide storage disorders. (1982) (195)
- Cathepsin D Deficiency Induces Lysosomal Storage with Ceroid Lipofuscin in Mouse CNS Neurons (2000) (188)
- Differences in the endosomal distributions of the two mannose 6- phosphate receptors (1993) (185)
- Cloning and expression of human arylsulfatase A. (1989) (175)
- Normal Lysosomal Morphology and Function in LAMP-1-deficient Mice* (1999) (172)
- 1.3 A structure of arylsulfatase from Pseudomonas aeruginosa establishes the catalytic mechanism of sulfate ester cleavage in the sulfatase family. (2001) (156)
- Processing of human cathepsin D in lysosomes in vitro. (1985) (153)
- Sequence determinants directing conversion of cysteine to formylglycine in eukaryotic sulfatases (1999) (144)
- Ultrastructural localization of the mannose 6-phosphate receptor in rat liver (1984) (143)
- Evidence for lysosomal enzyme recognition by human fibroblasts via a phosphorylated carbohydrate moiety. (1978) (138)
- Lysosomal enzyme precursors in human fibroblasts. Activation of cathepsin D precursor in vitro and activity of beta-hexosaminidase A precursor towards ganglioside GM2. (1982) (138)
- Heparan sulfate 6-O-endosulfatases: discrete in vivo activities and functional co-operativity. (2006) (137)
- Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId. (2002) (132)
- Conversion of cysteine to formylglycine: a protein modification in the endoplasmic reticulum. (1997) (128)
- Deficiency of UDP-N-acetylglucosamine:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase in organs of I-cell patients. (1982) (127)
- Carbohydrate deficient glycoprotein syndrome type IV: deficiency of dolichyl‐P‐Man:Man5 GlcNAc2‐PP‐dolichyl mannosyltransferase (1999) (126)
- A New Type of Carbohydrate-deficient Glycoprotein Syndrome Due to a Decreased Import of GDP-fucose into the Golgi* (1999) (124)
- LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice. (2003) (123)
- Processing of the phosphorylated recognition marker in lysosomal enzymes. Characterization and partial purification of a microsomal alpha-N-acetylglucosaminyl phosphodiesterase. (1981) (123)
- Functions of cathepsin K in bone resorption. Lessons from cathepsin K deficient mice. (2000) (122)
- A New Type of Congenital Disorders of Glycosylation (CDG-Ii) Provides New Insights into the Early Steps of Dolichol-linked Oligosaccharide Biosynthesis* (2003) (122)
- Phylogenetic conservation of arylsulfatases. cDNA cloning and expression of human arylsulfatase B. (1990) (121)
- Leukocyte adhesion deficiency II syndrome, a generalized defect in fucose metabolism☆☆☆ (1999) (121)
- Alternative mechanisms for trafficking of lysosomal enzymes in mannose 6-phosphate receptor-deficient mice are cell type-specific. (1999) (121)
- Neither type of mannose 6-phosphate receptor is sufficient for targeting of lysosomal enzymes along intracellular routes (1996) (115)
- Posttranslational Formation of Formylglycine in Prokaryotic Sulfatases by Modification of Either Cysteine or Serine* (1998) (114)
- Overlapping functions of lysosomal acid phosphatase (LAP) and tartrate-resistant acid phosphatase (Acp5) revealed by doubly deficient mice. (2001) (114)
- Structure of the arylsulfatase A gene. (1990) (112)
- Involvement of Nitric Oxide Released from Microglia–Macrophages in Pathological Changes of Cathepsin D-Deficient Mice (2001) (111)
- Is movement of mannose 6-phosphate-specific receptor triggered by binding of lysosomal enzymes? (1987) (108)
- The Leucine-based Sorting Motifs in the Cytoplasmic Domain of the Invariant Chain Are Recognized by the Clathrin Adaptors AP1 and AP2 and their Medium Chains* (1999) (106)
- Primary cultures of rat hepatocytes synthesize fibronectin. (1979) (104)
- A novel protein modification generating an aldehyde group in sulfatases: its role in catalysis and disease (1998) (103)
- Carbohydrate-deficient glycoprotein syndrome type V: deficiency of dolichyl-P-Glc:Man9GlcNAc2-PP-dolichyl glucosyltransferase. (1998) (103)
- Disease model: LAMP-2 enlightens Danon disease. (2001) (101)
- In vitro binding of plasma membrane-coated vesicle adaptors to the cytoplasmic domain of lysosomal acid phosphatase. (1993) (100)
- Amyloidogenic Processing of Human Amyloid Precursor Protein in Hippocampal Neurons Devoid of Cathepsin D* (1996) (96)
- A general binding mechanism for all human sulfatases by the formylglycine-generating enzyme. (2006) (96)
- The 46-kDa Mannose 6-Phosphate Receptor Contains Multiple Binding Sites for Clathrin Adaptors* (1997) (96)
- Crystal structure of an enzyme-substrate complex provides insight into the interaction between human arylsulfatase A and its substrates during catalysis. (2001) (95)
- Molecular basis of multiple sulfatase deficiency, mucolipidosis II/III and Niemann-Pick C1 disease - Lysosomal storage disorders caused by defects of non-lysosomal proteins. (2009) (93)
- An alternative hypothesis of cellular transport of lysosomal enzymes in fibroblasts. Effect of inhibitors of lysosomal enzyme endocytosis on intra- and extra-cellular lysosomal enzyme activities. (1978) (93)
- Sanfilippo syndrome type C: deficiency of acetyl-CoA:alpha-glucosaminide N-acetyltransferase in skin fibroblasts. (1978) (93)
- Identification of novel lysosomal matrix proteins by proteome analysis (2005) (93)
- Phosphorylated oligosaccharides in lysosomal enzymes: identification of alpha-N-acetylglucosamine(1)phospho(6)mannose diester groups. (1980) (92)
- Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VI. (1996) (91)
- Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells. (1989) (91)
- Arylsulfatase from Klebsiella pneumoniae Carries a Formylglycine Generated from a Serine* (1998) (88)
- Sanfilippo disease type D: deficiency of N-acetylglucosamine-6-sulfate sulfatase required for heparan sulfate degradation. (1980) (88)
- Biogenesis of lysosomal membranes (1994) (87)
- Involvement of Cathepsin E in Exogenous Antigen Processing in Primary Cultured Murine Microglia* (2002) (87)
- Autophagic Vacuoles with Sarcolemmal Features Delineate Danon Disease and Related Myopathies (2005) (85)
- Mannose 6-phosphate/insulin-like growth factor II receptor: distinct binding sites for mannose 6-phosphate and insulin-like growth factor II. (1988) (83)
- Synthesis of phosphorylated recognition marker in lysosomal enzymes is located in the cis part of Golgi apparatus. (1982) (81)
- Carbohydrate-free carboxypeptidase Y is transferred into the lysosome-like yeast vacuole. (1982) (81)
- Binding of AP2 to Sorting Signals Is Modulated by AP2 Phosphorylation* (2001) (79)
- Mannose 6-phosphate/insulin-like growth factor II-binding proteins in human serum and urine. Their relation to the mannose 6-phosphate/insulin-like growth factor II receptor. (1988) (78)
- Sulfatases, Trapping of the Sulfated Enzyme Intermediate by Substituting the Active Site Formylglycine * (1998) (78)
- Deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik. (2004) (77)
- Synthesis and processing of alpha-galactosidase A in human fibroblasts. Evidence for different mutations in Fabry disease. (1987) (75)
- Targeted Disruption of the Mouse Phosphomannomutase 2 Gene Causes Early Embryonic Lethality (2006) (74)
- Mannose 6-phosphate/insulin like growth factor II receptor: the two types of ligands bind simultaneously to one receptor at different sites. (1988) (73)
- The human SUMF1 gene, required for posttranslational sulfatase modification, defines a new gene family which is conserved from pro- to eukaryotes. (2003) (72)
- Phylogenetic conservation of cysteine proteinases. Cloning and expression of a cDNA coding for human cathepsin S. (1992) (71)
- Molecular Characterization of the Human Cα-formylglycine-generating Enzyme* (2005) (70)
- Differential involvement of the extracellular 6-O-endosulfatases Sulf1 and Sulf2 in brain development and neuronal and behavioural plasticity (2008) (69)
- The evolutionary conservation of a novel protein modification, the conversion of cysteine to serinesemialdehyde in arylsulfatase from Volvox carteri. (1996) (69)
- Amino Acid Residues Forming the Active Site of Arylsulfatase A (1999) (67)
- Mu 1A deficiency induces a profound increase in MPR300/IGF-II receptor internalization rate. (2001) (67)
- Subcellular location of two enzymes involved in the synthesis of phosphorylated recognition markers in lysosomal enzymes. (1981) (66)
- Quantitative aspects of pinocytosis and the intracellular fate of N-acetyl-alpha-D-glucosaminidase in Sanfilippo B fibroblasts. (1974) (65)
- Neutral endopeptidase-24.11 (enkephalinase). Biosynthesis and localization in human fibroblasts. (1987) (64)
- Golgi GDP-fucose Transporter-deficient Mice Mimic Congenital Disorder of Glycosylation IIc/Leukocyte Adhesion Deficiency II* (2007) (64)
- Quantitation of Mr 46000 and Mr 300000 mannose 6-phosphate receptors in human cells and tissues. (1991) (63)
- Metabolism of sulfated glycosaminoglycans in cultivated bovine arterial cells. I. Characterization of different pools of sulfated glycosaminoglycans. (1975) (63)
- Morquio syndrome (mucopolysaccharidosis IV B) associated with beta-galactosidase deficiency. Report of two cases. (1980) (62)
- Lysosomal enzyme precursors in coated vesicles derived from the exocytic and endocytic pathways (1987) (61)
- Mutations in the arylsulfatase A pseudodeficiency allele causing metachromatic leukodystrophy. (1991) (61)
- Juvenile and adult metachromatic leukodystrophy: partial restoration of arylsulfatase A (cerebroside sulfatase) activity by inhibitors of thiol proteinases. (1983) (60)
- Neurocognitive and Psychotiform Behavioral Alterations and Enhanced Hippocampal Long-Term Potentiation in Transgenic Mice Displaying Neuropathological Features of Human α-Mannosidosis (2005) (60)
- Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): six unique arylsulfatase B gene alleles causing variable disease phenotypes. (1994) (60)
- Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). An intermediate clinical phenotype caused by substitution of valine for glycine at position 137 of arylsulfatase B. (1991) (59)
- On the effects of weak bases and monensin on sorting and processing of lysosomal enzymes in human cells. (1987) (59)
- Expression of the two mannose 6-phosphate receptors is spatially and temporally different during mouse embryogenesis. (1992) (59)
- Two new arylsulfatase A (ARSA) mutations in a juvenile metachromatic leukodystrophy (MLD) patient. (1991) (59)
- Enhanced breakdown of arylsulfatase A in multiple sulfatase deficiency. (1982) (58)
- Genetic heterogeneity of steroid sulfatase deficiency revealed with cDNA for human steroid sulfatase. (1987) (57)
- Efficacy of enzyme replacement therapy in alpha-mannosidosis mice: a preclinical animal study. (2004) (56)
- Granzyme-mediated Cytotoxicity Does Not Involve the Mannose 6-Phosphate Receptors on Target Cells* (2004) (56)
- UDP-N-acetylglucosamine:lysosomal enzyme precursor N-acetylglucosamine-1-phosphotransferase. Partial purification and characterization of the rat liver Golgi enzyme. (1982) (55)
- Deficiency of dolichyl-P-Man:Man7GlcNAc2-PP-dolichyl mannosyltransferase causes congenital disorder of glycosylation type Ig. (2002) (55)
- AP‐1 and AP‐3 Mediate Sorting of Melanosomal and Lysosomal Membrane Proteins into Distinct Post‐Golgi Trafficking Pathways (2008) (54)
- 46-kDa mannose 6-phosphate-specific receptor: biosynthesis, processing, subcellular location and topology. (1987) (54)
- Biosynthesis and transport of lysosomal enzymes in human monocytes and macrophages. Effects of ammonium chloride, zymosan and tunicamycin. (1983) (53)
- Cathepsin D and beta-hexosaminidase synthesized in the presence of 1-deoxynojirimycin accumulate in the endoplasmic reticulum. (1984) (53)
- Effect of monensin on intracellular transport and receptor-mediated endocytosis of lysosomal enzymes. (1984) (53)
- Molecular forms of beta-hexosaminidase and cathepsin D in serum and urine of healthy subjects and patients with elevated activity of lysosomal enzymes. (1983) (52)
- Residues critical for formylglycine formation and/or catalytic activity of arylsulfatase A. (1998) (51)
- In vitro mutagenesis of potential N-glycosylation sites of arylsulfatase A. Effects on glycosylation, phosphorylation, and intracellular sorting. (1992) (50)
- Synthesis and processing of arylsulfatase A in human skin fibroblasts. (1982) (50)
- Cathepsin D precursors in clathrin-coated organelles from human fibroblasts (1985) (49)
- Posttranslational Modification of Serine to Formylglycine in Bacterial Sulfatases (2003) (47)
- The tyrosine motifs of Lamp 1 and LAP determine their direct and indirect targetting to lysosomes. (2002) (47)
- Structure of the human arylsulfatase B gene. (1993) (47)
- Abnormal synthesis of mannose 1-phosphate derived carbohydrates in carbohydrate-deficient glycoprotein syndrome type I fibroblasts with phosphomannomutase deficiency. (1998) (46)
- Proteolytic processing of cathepsin D in prelysosomal organelles. (1994) (44)
- 46-kDa mannose 6-phosphate-specific receptor: purification, subunit composition, chemical modification. (1987) (44)
- Receptor-mediated endocytosis of proteoglycans by human fibroblasts involves recognition of the protein core. (1983) (43)
- Synthesis and transport of lysosomal acid phosphatase in normal and I-cell fibroblasts. (1985) (43)
- Partial purification and characterization of heparan sulfate specific endoglucuronidase. (1976) (42)
- ERp44 Mediates a Thiol-independent Retention of Formylglycine-generating Enzyme in the Endoplasmic Reticulum* (2008) (42)
- Occurrence of tyrosine sulfate in proteins--a balance sheet. 1. Secretory and lysosomal proteins. (1990) (42)
- Effect of tunicamycin on transport of lysosomal enzymes in cultured skin fibroblasts. (1979) (41)
- Biosynthesis and maturation of arylsulfatase B in normal and mutant cultured human fibroblasts. (1983) (41)
- Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAs. (1992) (41)
- Mistargeting of lysosomal enzymes in M(r) 46,000 mannose 6-phosphate receptor-deficient mice is compensated by carbohydrate-specific endocytotic receptors. (1994) (40)
- Enhanced degradation of cathepsin D synthesized in the presence of the threonine analog beta-hydroxynorvaline. (1984) (40)
- Effects of brefeldin A on the endocytic route. Redistribution of mannose 6-phosphate/insulin-like growth factor II receptors to the cell surface. (1991) (39)
- The two mannose 6-phosphate receptors have almost identical subcellular distributions in U937 monocytes. (1988) (39)
- Transport and processing of lysosomal enzymes by smooth muscle cells and endothelial cells. (1981) (39)
- Identification of N-sulphated disaccharide units in heparin-like polysaccharides. (1979) (39)
- Studies on secretion and endocytosis of macromolecules by cultivated skin fibroblasts. Effects of anti-microtubular agents on secretion and endocytosis of lysosomal hydrolases and of sulphated glycosaminoglycans. (1978) (38)
- Evidence for degradation of heparan sulfate by endoglycosidases: glucosamine and hexuronic acid are reducing terminals of intracellular heparan sulfate from human skin fibroblasts. (1976) (38)
- Deficiency of arylsulfatase B in 2 brothers aged 40 and 38 years. (Maroteaux‐Lamy syndrome, type B) (1979) (37)
- Spontaneous Mucolipidosis in a Cat: An Animal Model of Human I-Cell Disease (1996) (37)
- Quaternary structure of the Mr 46,000 mannose 6-phosphate specific receptor: effect of ligand, pH, and receptor concentration on the equilibrium between dimeric and tetrameric receptor forms. (1990) (37)
- Metabolism of sulfated glycosaminoglycans in rat hepatocytes. Synthesis of heparan sulfate and distribution into cellular and extracellular pools. (1980) (36)
- Structure of the human lysosomal acid phosphatase gene. (1989) (36)
- Glycosylation and phosphorylation of arylsulfatase A. (1994) (36)
- Insulin-like growth factors I and II stimulate endocytosis but do not affect sorting of lysosomal enzymes in human fibroblasts. (1990) (36)
- Defective Oligomerization of Arylsulfatase A as a Cause of Its Instability in Lysosomes and Metachromatic Leukodystrophy* (2002) (35)
- Uptake of hyaluronate by cultured cells. (1977) (35)
- Molecular recognition and targeting of lysosomal proteins. (1991) (35)
- Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation Published, JLR Papers in Press, September 21, 2005. DOI 10.1194/jlr.M500131-JLR200 (2005) (35)
- Biosynthesis of acid α‐glucosidase in late‐onset forms of glycogenosis type II (Pompe's disease) (1982) (35)
- The Normal Phenotype of Pmm1-Deficient Mice Suggests that Pmm1 Is Not Essential for Normal Mouse Development (2006) (34)
- Characterization of Posttranslational Formylglycine Formation by Luminal Components of the Endoplasmic Reticulum* (2001) (34)
- Tilorone acts as a lysosomotropic agent in fibroblasts. (1984) (34)
- High residual arylsulfatase A (ARSA) activity in a patient with late-infantile metachromatic leukodystrophy. (1993) (33)
- Cell surface-associated lysosomal enzymes in cultured human skin fibroblasts. (1979) (33)
- Sanfilippo B disease: serum assays for detection of homozygous and heterozygous individuals in three families. (1973) (33)
- N-Acetylglucosamine-6-sulfate sulfatase from human urine. (1979) (33)
- Late‐onset metachromatic leukodystrophy: Molecular pathology in two siblings (1992) (32)
- Mannose 6-phosphate-specific receptor is a transmembrane protein with a C-terminal extension oriented towards the cytosol. (1985) (32)
- Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis. (1999) (32)
- Locating the anomalous scatterer substructures in halide and sulfur phasing. (2003) (31)
- Endocytosis of lysosomal enzymes by non-parenchymal rat liver cells. Comparative study of lysosomal-enzyme uptake by hepatocytes and non-parenchymal liver cells. (1979) (31)
- Recognition of human urine alpha-N-acetylglucosaminidase by rat hepatocytes. Involvement of receptors specific for galactose, mannose 6-phosphate and mannose. (1979) (31)
- Tartrate-inhibitable acid phosphatase. Purification from placenta, characterization and subcellular distribution in fibroblasts. (1984) (31)
- Mannose 6-phosphate receptors (MPR 300 and MPR 46) from a teleostean fish (trout). (1999) (30)
- Endocytosis of sulphated proteoglycans by cultured skin fibroblasts. (1978) (30)
- Juvenile form of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). A C-terminal extension causes instability but increases catalytic efficiency of arylsulfatase B. (1994) (30)
- Carbohydrate-deficient glycoprotein syndrome type 1: correction of the glycosylation defect by deprivation of glucose or supplementation of mannose (1998) (30)
- Structure of the mouse arylsulfatase A gene and cDNA. (1994) (30)
- Molecular characterization of the hypothetical 66.3‐kDa protein in mouse: Lysosomal targeting, glycosylation, processing and tissue distribution (2006) (29)
- Structural Requirements for Interactions between Leucine-sorting Signals and Clathrin-associated Adaptor Protein Complex AP3* (2002) (29)
- Congenital disorder of glycosylation-Ic: case report and genetic defect. (2000) (29)
- Proteolysis of IGFBPs by cathepsin D in vitro and in cathepsin D-deficient mice. (1995) (29)
- Differently labelled glucosamine-precursor pools for the biosynthesis of hyaluronate and heparan sulfate. (1973) (29)
- The carboxy-terminal peptides of 46 kDa and 300 kDa mannose 6-phosphate receptors share partial sequence homology and contain information for sorting in the early endosomal pathway. (1997) (29)
- Localization of sulfated glycosaminoglycans within cell nuclei by high-resolution autoradiography. (1976) (29)
- Mr 46,000 mannose 6-phosphate receptor. The role of histidine and arginine residues for binding of ligand. (1991) (29)
- Synthesis, transport and processing of cathepsin C in Morris hepatoma 7777 cells and rat hepatocytes. (1985) (29)
- Expression and processing of the activin-A/erythroid differentiation factor precursor: a member of the transforming growth factor-beta superfamily. (1990) (29)
- Expression, Localization, Structural, and Functional Characterization of pFGE, the Paralog of the Cα-Formylglycine-generating Enzyme* (2005) (28)
- Lysosomal acid phosphatase is internalized via clathrin-coated pits. (1992) (28)
- The polarized epithelia‐specific μ1B‐adaptin complements μ1A‐deficiency in fibroblasts (2002) (28)
- An N‐acetylgalactosamine‐4‐sulfatase mutation (ΔG238) results in a severe Maroteaux‐Lamy phenotype (1992) (27)
- A single origin for the most frequent mutation causing late infantile metachromatic leucodystrophy. (1994) (27)
- Molecular basis of late infantile metachromatic leukodystrophy in the Habbanite Jews (1995) (27)
- Synthesis and stability of arylsulfatase A and B in fibroblasts from multiple sulfatase deficiency. (1985) (27)
- Synthesis of a truncated Mr 46,000 mannose 6-phosphate receptor that is secreted and retains ligand binding. (1989) (26)
- Metabolism of sulfated glycosaminoglycans in cultivated bovine arterial cells. II. Quantitative studies on the uptake of 35SO4-labeled proteoglycans. (1975) (26)
- Crystal Structure of Human pFGE, the Paralog of the Cα-formylglycine-generating Enzyme* (2005) (26)
- Proteolytic degradation of glutamate decarboxylase mediates disinhibition of hippocampal CA3 pyramidal cells in cathepsin D‐deficient mice (2005) (26)
- Steroid sulfatase. Biosynthesis and processing in normal and mutant fibroblasts. (1986) (25)
- Tail-specific antibodies that block return of 46,000 M(r) mannose 6- phosphate receptor to the trans-Golgi network (1993) (25)
- Translational control of arylsulfatase A expression in mouse testis. (1994) (25)
- Immuno-electron microscopical demonstration of lysosomes in human blood platelets and megakaryocytes using anti-cathepsin D. (1985) (25)
- Glycosylation of the Mr 46,000 mannose 6-phosphate receptor. Effect on ligand binding, stability, and conformation. (1991) (25)
- Simultaneous redistribution of mannose 6-phosphate and transferrin receptors by insulin-like growth factors and phorbol ester. (1992) (25)
- Regulation of mannose 6-phosphate/insulin-like growth factor II receptor distribution by activators and inhibitors of protein kinase C. (1990) (24)
- Characterization of phosphorylation sites in the cytoplasmic domain of the 300 kDa mannose-6-phosphate receptor. (1993) (23)
- [Identification of mutations in the arylsulfatase B gene in Russian mucopolysaccharidosis type VI patients]. (2000) (23)
- Four monoclonal antibodies inhibit the recognition of arylsulphatase A by the lysosomal enzyme phosphotransferase. (1994) (22)
- Cloning and sequence analysis of human guanidinoacetate N-methyltransferase cDNA. (1995) (22)
- Mouse cathepsin D gene: molecular organization, characterization of the promoter, and chromosomal localization. (1994) (22)
- Initiation of altered heparan sulphate on beta-D-xyloside in rat hepatocytes. (1981) (22)
- Biosynthesis and maturation of alpha-N-acetylglucosaminidase in normal and Sanfilippo B-fibroblasts. (1984) (21)
- High degree of homology between primary structure of human lysosomal acid phosphatase and human prostatic acid phosphatase. (1989) (21)
- Substrate specificity of a heparan sulfate-degrading endoglucuronidase from human placenta. (1979) (21)
- Identification of formylglycine in sulfatases by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry. (2003) (21)
- The ligand-binding conformation of Mr 46,000 mannose 6-phosphate-specific receptor. Acquisition of binding activity during in vitro synthesis. (1989) (20)
- Adhesion of human skin fibroblasts. Effect of tetravalent, divalent and monovalent concanavalin A. (1978) (20)
- Mucolipidosis type II in a domestic shorthair cat. (1996) (20)
- Phosphorylation of lysosomal enzymes in fibroblasts. Marked deficiency of N-acetylglucosamine-1-phosphotransferase in fibroblasts of patients with mucolipidosis III. (1982) (20)
- Complex arylsulfatase A alleles causing metachromatic leukodystrophy (1994) (20)
- Association of the precursor of cathepsin D with coated membranes. Kinetics and carbohydrate processing. (1987) (19)
- Inhibition by cyanate of the processing of lysosomal enzymes. (1983) (19)
- Identification of the Putative Mannose 6-phosphate Receptor Protein (MPR 300) in the Invertebrate unio (1999) (19)
- A new biochemical subtype of the Sanfilippo syndrome: characterization of the storage material in cultured fibroblasts of Sanfilippo C patients. (1978) (19)
- Two allelic forms of human arylsulfatase A with different numbers of asparagine-linked oligosaccharides. (1983) (19)
- Arylsulfatase A pseudodeficiency: a common polymorphism which is associated with a unique haplotype. (1994) (19)
- Comparison of biosynthesis and subcellular distribution of lysozyme and lysosomal enzymes in U937 monocytes. (1985) (18)
- Two novel frameshift mutations causing premature stop codons in a patient with the severe form of Maroteaux‐Lamy syndrome (1996) (18)
- Sanfilippo disease type B: presence of material cross reacting with antibodies against alpha-N-acetylglucosaminidase. (1976) (18)
- Conserved cassette structure of vertebrate Mr 300 kDa mannose 6-phosphate receptors: partial cDNA sequence of fish MPR 300. (2000) (18)
- The oligomeric state of 46-kDa mannose 6-phosphate receptor does not change upon intracellular recycling and binding of ligands. (1996) (17)
- Alteration in pancreatic immunoreactivity of insulin-like growth factor (IGF)-binding protein (IGFBP)-6 and in intracellular degradation of IGFBP-3 in fibroblasts of IGF-II receptor/IGF-II-deficient mice. (1999) (17)
- Lysosomal acid phosphatase is not involved in the dephosphorylation of mannose 6-phosphate containing lysosomal proteins. (1992) (17)
- Isolation and analysis of the human 46-kDa mannose 6-phosphate receptor gene. (1991) (17)
- Conditions for a reliable application of the lysoplate method in the determination of lysozyme. (1987) (16)
- Natural killer cell-mediated cytotoxicity does not depend on recognition of mannose 6-phosphate residues. (1985) (16)
- Heterogeneity in late-onset metachromatic leukodystrophy. Effect of inhibitors of cysteine proteinases. (1986) (16)
- Effects of differentiation-inducing agents on synthesis, maturation and secretion of cathepsin D in U937 and HL-60 cells. (1987) (16)
- Mutational analysis of disulfide bridges in the Mr 46,000 mannose 6-phosphate receptor. Localization and role for ligand binding. (1991) (16)
- The Non-catalytic N-terminal Extension of Formylglycine-generating Enzyme Is Required for Its Biological Activity and Retention in the Endoplasmic Reticulum* (2008) (16)
- Restoration of arylsulphatase B activity in human mucopolysaccharidosis-type-VI fibroblasts by retroviral-vector-mediated gene transfer. (1991) (16)
- Assembly of the ligand-binding conformation of Mr 46,000 mannose 6- phosphate-specific receptor takes place before reaching the Golgi complex (1990) (16)
- Restoration of arylsulphatase A activity in human-metachromatic-leucodystrophy fibroblasts via retroviral-vector-mediated gene transfer. (1991) (16)
- Low temperature blocks transport and sorting of cathepsin D in fibroblasts. (1988) (16)
- Sulfated oligosaccharides in human lysosomal enzymes. (1987) (16)
- Epithelial rat liver cells have cell surface receptors recognizing a phosphorylated carbohydrate on lysosomal enzymes. (1978) (15)
- Multiple sulfatase deficiency: degradation of arylsulfatase A and B after endocytosis in fibroblasts. (1985) (15)
- Insulin-like growth factor II overexpression does not affect sorting of lysosomal enzymes in NIH-3T3 cells. (1991) (14)
- Targeting of lysosomal acid phosphatase with altered carbohydrate. (1989) (14)
- Synthesis and stability of steroid sulfatase in fibroblasts from multiple sulfatase deficiency. (1988) (14)
- Dephosphorylation of the mannose-6-phosphate recognition marker is localized in later compartments of the endocytic route. Identification of purple acid phosphatase (uteroferrin) as the candidate phosphatase. (1996) (14)
- Metabolism of cell surface-associated sulfated glycosaminoglycans in cultured human smooth muscle cells. (1980) (14)
- Biosynthesis of secreted beta-hexosaminidase in Tetrahymena thermophila. A comparison of the wild type with a secretory mutant. (1988) (14)
- Immunoelectron-microscopic study on the location of fibronectin in human fibroblast cultures. (1982) (14)
- Sanfilippo's disease type A: sulfamidase activity in peripheral leukocytes of normal, heterozygous and homozygous individuals. (1977) (13)
- Effect of tunicamycin on the metabolism of low-density lipoproteins by control and low-density-lipoprotein-receptor-deficient human skin fibroblasts. (1980) (13)
- Molecular cloning of the mouse 46-kDa mannose 6-phosphate receptor (MPR 46). (1991) (13)
- Endocytosis of beta-N-acetylglucosaminidase from sections of mucolipidosis-II and-III fibroblasts by non-parenchymal rat liver cells. (1979) (13)
- Processing and transport of lysosomal enzymes in human monocyte line U937. (1984) (13)
- Regulation of heparan sulphate metabolism by adenosine 3':5'-cyclic monophosphate in hepatocytes in culture. (1980) (12)
- Characterization of dermatan sulfate in mucopolysaccharidosis VI. Evidence for the absence of hyaluronidase-like enzymes in human skin fibroblasts. (1980) (12)
- Oligosaccharides in lysosomal enzymes. Distribution of high-mannose and complex oligosaccharides in cathepsin D and beta-hexosaminidase. (1981) (12)
- Identification of the Putative Mannose 6-Phosphate Receptor (MPR 46) Protein in the Invertebrate Mollusc (2002) (12)
- Characterization of glycosaminoglycans stored in mucopolysaccharidosis III A: evidence for a generally occuring degradation of heparan sulfate by endoglycosidases. (1977) (12)
- The ‘cation‐dependent’ mannose 6‐phosphate receptor binds ligands in the absence of divalent cations (1988) (12)
- Molecular cloning of the mouse lysosomal acid phosphatase. (1991) (11)
- Human placenta alpha-N-acetylglucosaminidase: purification, characterization and demonstration of multiple recognition forms. (1978) (11)
- Sequential detection of antigens in Western blots with differently colored products. (1986) (11)
- Molecular forms of cathepsin D in coated vesicle preparations. (1983) (11)
- Physical properties and biological activities of two forms of alpha-N-acetylglucosaminidase from bovine spleen. (1974) (11)
- The 46 kDa mannose-6-phosphate receptor contains a signal for basolateral sorting within the 19 juxtamembrane cytosolic residues. (1997) (11)
- Paralog of the formylglycine‐generating enzyme – retention in the endoplasmic reticulum by canonical and noncanonical signals (2008) (11)
- Localization of lysosomal acid phosphatase mRNA in mouse tissues. (1992) (10)
- Impaired phosphorylation of lysosomal enzymes in fibroblasts of patients with mucolipidosis III. (1982) (10)
- Function and Properties of Chimeric MPR 46-MPR 300 Mannose 6-Phosphate Receptors* (2000) (10)
- Human alpha-N-acetylglucosaminidase. 1. Purification and properties. (1977) (9)
- Cell type dependent inhibition of transport of cathepsin D in HepG2 cells and fibroblasts exposed to deoxy-manno-nojirimycin and deoxynojirimycin. (1985) (9)
- Effect of lectins on endocytosis and secretion of lysosomal enzymes by cultured fibroblasts. (1979) (9)
- Mutations affecting transport and stability of lysosomal enzymes. (1987) (8)
- Phosphorylation of arylsulphatase A occurs through multiple interactions with the UDP-N-acetylglucosamine-1-phosphotransferase proximal and distal to its retrieval site by the KDEL receptor. (1999) (8)
- Localisation of acetyl-CoA: alpha-glucosaminide N-acetyltransferase in microsomes and lysosomes of rat liver. (1981) (8)
- Human alpha-n-acetylglucosaminidase. 2. Activity towards natural substrates and multiple recognition forms. (1977) (8)
- Homologous low density lipoprotein does not affect proteoglycan metabolism of cultured skin fibroblasts and arterial smooth muscle cells. (1980) (8)
- Lysosomal membrane proteins do not bind to mannose-6-phosphate-specific receptors. (1986) (8)
- Late infantile metachromatic leukodystrophy in Israel. (1994) (8)
- Specific binding of sulfated proteoglycans to concanavalin A - Sepharose 4B. (1978) (7)
- 14C and 35S-SO4 metabolism of acid glycosaminoglycans in bovine heart valves. (1973) (7)
- Unimpaired allorejection of cells deficient for the mannose 6-phosphate receptors Mpr300 and Mpr46. (2004) (7)
- Rapid equilibrium between monomeric, dimeric and tetrameric forms of the 46-kDa mannose 6-phosphate receptor at 37 degrees C. Possible relation to the function of the receptor. (1990) (7)
- The sanfilippo B corrective factor: a N-acetyl-alpha-D-glucosamindiase. (1972) (7)
- Brefeldin A affects the cellular distribution of endocytic receptors differentially. (1992) (7)
- Genetics of metachromatic leukodystrophy. (1994) (6)
- Prenatal diagnosis of morquio's disease type a (n‐acetylgalactosamine 6‐sulphate sulphatase deficiency) (1982) (6)
- Prostaglandins and fibroblast functions in vitro. (1979) (6)
- Mannose 6-phosphate specific receptors: structure and function. (1989) (6)
- Lysosomal glycogen storage disease without deficiency of acid alpha-glucosidase. (1989) (6)
- Effect of lectins on the metabolism of sulfated glycosaminolycans in cultured fibroblasts. (1979) (6)
- A 3H-labelled trisaccharide from heparin as substrate for acetyl-CoA: 2-amino-2-deoxy-alpha-D-glucoside N-acetyltransferase. (1980) (6)
- Degradation of even-numbered reduced and non-reduced hyaluronate oligosaccharides with D-glucuronic acid or N-acetyl-D-glucosamine as non-reducing terminal by chondroitin ABC and AC lyases. (1979) (6)
- Serum α-N-acetylglucosaminidase: Determination, Characterization, and Corrective Activity in Sanfilippo B Fibroblasts (1975) (5)
- Biosynthesis of acid alpha-glucosidase in late-onset forms of glycogenosis type II (Pompe's disease). (1982) (5)
- Studies on the chemistry of arterial wall, XVI. Topochemical studies on the glycoasaminoglycan and lipid metabolism in bovine arterial tissue. (1973) (5)
- Human tartrate-inhibitable lysosomal acid phosphatase. Purification, characterization, biosynthesis and intracellular transport. (1986) (4)
- Studies of the (+)-catechin action on the metabolism of bovine arterial tissue. (1972) (4)
- Storage of mannose-containing material in cultured human mannosidosis cells and metabolic correction by pig kidney alpha-mannosidase. (1976) (4)
- Prostaglandins, cyclic nucleotides and glycosaminoglycan biosynthesis in cultured fibroblasts. (1978) (3)
- Synthesis of lysosomal alpha-mannosidase in normal and mannosidosis fibroblasts. (1983) (3)
- Secretion of beta-hexosaminidase by cultured human skin fibroblasts. Kinetics, effect of temperature, cell density, serum concentration and pH. (1978) (2)
- Receptor-dependent transport of lysosomal enzymes. (1988) (2)
- Inhibition of pinocytosis by cytochalasin B. Decrease in intracellular lysosomal-enzyme activities and increased storage of glycosaminoglycans. (1974) (2)
- Stimulation of heparan sulphate synthesis in cultured rat hepatocytes by (+)-catechin. (1981) (2)
- Mannose 6-phosphate/insulin-like growth factor II receptor in I-cell disease fibroblasts: increased synthesis and defective regulation of cell surface expression. (1992) (1)
- Lymphocyte histochemistry--a screening test for early detection of storage disorders? (1983) (1)
- Isolation and characterization of phosphorylated oligosaccharides from alpha-N-acetylglucosaminidase that are recognized by cell-surface receptors. (1979) (1)
- Serum alpha-N-acetylglucosaminidase: determination, characterization, and corrective activity in Sanifilippo B fibroblasts. (1975) (1)
- [Biochemistry and function of glycosaminoglycans of the skin]. (1976) (1)
- [Quantitative aspects of mucopolysaccharide degradation correction in Sanfilippo-B-fibroblasts due to N-acetyl-alpha-D-glucosaminidase]. (1972) (0)
- STRUCTURE‐FUNCTION RELATIONSHIP FOR LYSOSOMAL ENZYMES (2007) (0)
- [Respiratory functions of 6 to 10 year old children with low birth weight (author's transl)]. (1974) (0)
- Structure–function relationship for lysosomal enzymes (2007) (0)
- [Mannose-6-phosphate receptors: their role in the transport of lysosomal proteins]. (1990) (0)
- [Mucopolysaccharidosis I-S (Scheie's disease) (author's transl)]. (1979) (0)
- [Carbon-replica-cytochemistry--a method for localization of cell surface components (author's transl)]. (1982) (0)
- [A new type of mucopolysaccharidosis]. (1972) (0)
- [Topochemical differences in the metabolism of glyco-aminoglycanes in the bovine aorta]. (1972) (0)
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