William S. Sly
American biochemist and molecular biologist
William S. Sly's AcademicInfluence.com Rankings
Download Badge
Biology
William S. Sly's Degrees
- PhD Biochemistry University of California, Berkeley
- Bachelors Chemistry University of California, Berkeley
Why Is William S. Sly Influential?
(Suggest an Edit or Addition)According to Wikipedia, William S. Sly is an internationally known physician and scientist who, except for sabbatical years at Oxford and Stanford, spent his entire academic career in St. Louis. Following M.D. training at Saint Louis University School of Medicine, he trained in internal medicine at Washington University in St. Louis and in research laboratories at the NIH, in Paris, and in Madison, Wisconsin. He then joined the faculty at Washington University, where he directed the division of medical genetics for 20 years. In 1984, he was recruited to St. Louis University School of Medicine and appointed Alice A. Doisy Professor and chairman of the Edward A. Doisy Department of Biochemistry and Molecular Biology. He chaired that department for 26 years. In February 2007, he was also named the inaugural holder of the James B. and Joan C. Peters Endowed Chair in Biochemistry and Molecular Biology. He became an emeritus professor in July 2014.
William S. Sly's Published Works
Published Works
- Lysosomes in biology and pathology (1969) (1453)
- Chloroquine inhibits lysosomal enzyme pinocytosis and enhances lysosomal enzyme secretion by impairing receptor recycling (1980) (642)
- Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts. (1977) (435)
- The human cation-independent mannose 6-phosphate receptor. Cloning and sequence of the full-length cDNA and expression of functional receptor in COS cells. (1988) (213)
- Phosphomannosyl-enzyme receptors in rat liver. Subcellular distribution and role in intracellular transport of lysosomal enzymes. (1980) (184)
- Enzymatic identification of mannose 6-phosphate on the recognition marker for receptor-mediated pinocytosis of beta-glucuronidase by human fibroblasts. (1979) (157)
- Phosphohexosyl recognition is a general characteristic of pinocytosis of lysosomal glycosidases by human fibroblasts. (1977) (153)
- The insulin-like growth factor II (IGF-II)/mannose 6-phosphate receptor mediates IGF-II-induced motility in human rhabdomyosarcoma cells. (1992) (136)
- Treatment of MPS VII (Sly disease) by allogeneic BMT in a female with homozygous A619V mutation (1998) (107)
- Beta-glucuronidase binding to human fibroblast membrane receptors. (1980) (106)
- Distinctive regulation of the functional linkage between the human cation-independent mannose 6-phosphate receptor and GTP-binding proteins by insulin-like growth factor II and mannose 6-phosphate. (1990) (100)
- The role of glycosylation and phosphorylation in the expression of active human beta-glucuronidase. (1993) (81)
- Membrane‐bound carbonic anhydrase IV is expressed in the luminal plasma membrane of the human gallbladder epithelium (1996) (72)
- Structural studies of the phosphorylated high mannose-type oligosaccharides on human beta-glucuronidase. (1982) (71)
- Cloning and expression of the cDNA of chicken cation-independent mannose-6-phosphate receptor. (1995) (68)
- Binding of insulin-like growth factor II (IGF-II) by human cation-independent mannose 6-phosphate receptor/IGF-II receptor expressed in receptor-deficient mouse L cells. (1990) (67)
- Mutational analysis of a patient with mucopolysaccharidosis type VII, and identification of pseudogenes. (1993) (58)
- The regulation of fatty acid biosynthesis in human skin fibroblasts. (1973) (56)
- The overexpressed human 46-kDa mannose 6-phosphate receptor mediates endocytosis and sorting of beta-glucuronidase. (1990) (52)
- Overexpression rescues the mutant phenotype of L176F mutation causing beta-glucuronidase deficiency mucopolysaccharidosis in two Mennonite siblings. (1994) (51)
- Binding of phosphorylated oligosaccharides to immobilized phosphomannosyl receptors. (1982) (50)
- Cloning, sequencing, and functional characterization of the murine 46-kDa mannose 6-phosphate receptor. (1991) (50)
- Expression of human cation-independent mannose 6-phosphate receptor cDNA in receptor-negative mouse P388D1 cells following gene transfer. (1988) (46)
- Demonstration of the heterozygous state for I-cell disease and pseudo-Hurler polydystrophy by assay of N-acetylglucosaminylphosphotransferase in white blood cells and fibroblasts. (1982) (43)
- Mucopolysaccharidosis IVA: identification of mutations and methylation study in GALNS gene (2004) (40)
- C-terminal processing of human beta-glucuronidase. The propeptide is required for full expression of catalytic activity, intracellular retention, and proper phosphorylation. (1993) (40)
- Correlation of structural features of phosphomannans with their ability to inhibit pinocytosis of human beta-glucuronidase by human fibroblasts. (1978) (39)
- Fibroblast receptor for lysosomal enzymes mediates pinocytosis of multivalent phosphomannan fragment (1980) (39)
- Intracellular traffic of the mannose 6-phosphate receptor and its ligands. (1987) (25)
- Four novel mutations in mucopolysaccharidosis type VII including a unique base substitution in exon 10 of the beta-glucuronidase gene that creates a novel 5'-splice site. (1995) (24)
- FORMATE METABOLISM. II. ENZYMATIC SYNTHESIS OF FORMYL PHOSPHATE AND FORMYL COENZYME A IN CLOSTRIDIUM CYLINDROSPORUM. (1963) (22)
- Adsorptive pinocytosis of phosphorylated oligosaccharides by human fibroblasts. (1982) (22)
- FORMATE METABOLISM. I. FORMYL COENZYME A, AN INTERMEDIATE IN THE FORMATE-DEPENDENT DECOMPOSITION OF ACETYL PHOSPHATE IN CLOSTRIDIUM KLUYVERI. (1963) (19)
- Enzyme replacement therapy: from concept to clinical practice (2002) (18)
- Human beta-glucuronidase pinocytosis and binding to the immobilized phosphomannosyl receptor. Effects of treatment of the enzyme with alpha-N-acetylglucosaminyl phosphodiesterase. (1983) (15)
- Divalent cation-dependent stimulation of ligand binding to the 46-kDa mannose 6-phosphate receptor correlates with divalent cation-dependent tetramerization. (1992) (15)
- Concanavalin A mediated uptake of enzymes by fibroblasts. (1978) (12)
- Lysosomal storage defects. (1985) (12)
- Efficient and persistent expression of β‐glucuronidase gene in CD34+ cells from human umbilical cord blood by retroviral vector (1998) (9)
- CELL-FREE PEPTIDE SYNTHESIS DEPENDENT UPON SYNTHETIC OLIGODEOXYNUCLEOTIDES. (1963) (8)
- Methylation patterns of entire human β‐glucuronidase gene locus: boundaries of methylation and general implications for frequent point mutations at CpG dinucleotides (2002) (6)
- What is genetic counseling? (1973) (3)
- Prospects for enzyme replacement for lysosomal storage diseases. (1981) (3)
- A new approach to develop mice models by targeted mutagenesis: production of a mucopolysccharidosis type VII mouse(Gustm(hE540A · mE536A)sly) doubly tolerant to human and mouse β‐glucuronidase (2002) (2)
- Mouse model of Morquio A syndrome (Galns‐/‐) produced by targeted disruption of the gene encoding N‐acetylgalactosamine‐6‐sulphate sulphatase (2002) (0)
- Molecular Pathophysiology of Intestinal Iron Transport in Haemochromatosis (2001) (0)
- Crystallization and preliminary crystallographic studies of human beta-glucuronidase. (1993) (0)
- Molecular analysis of a mild form of mucopolysaccharidosis VII: novel mutations and expression studies (2002) (0)
- Towards a new treatment: role of the International Morquio Registry (2002) (0)
- Missense models (Gustm(L175F)Sly, Gustm(E536A)sly, and Gustm(E536Q)sly) of murine mucopolysaccharidosis type VII produced by targeted mutagenesis (2002) (0)
This paper list is powered by the following services:
Other Resources About William S. Sly
What Schools Are Affiliated With William S. Sly?
William S. Sly is affiliated with the following schools: