Published in Biochem J on October 01, 1978
Enzymatic identification of mannose 6-phosphate on the recognition marker for receptor-mediated pinocytosis of beta-glucuronidase by human fibroblasts. Proc Natl Acad Sci U S A (1979) 2.28
The stability and aggregation properties of human liver acid beta-D-galactosidase. Biochem J (1981) 0.97
Human placental beta-galactosidase. Characterization of the dimer and complex forms of the enzyme. Biochem J (1992) 0.95
Purification and characterization of two forms of beta-D-galactosidase from rat epididymal luminal fluid: evidence for their role in the modification of sperm plasma membrane glycoprotein(s). Biochem J (1995) 0.86
Structure of the lysosomal neuraminidase-beta-galactosidase-carboxypeptidase multienzymic complex. Biochem J (1990) 0.83
Rat epididymal luminal fluid acid beta-D-galactosidase optimally hydrolyses glycoprotein substrate at neutral pH. Biochem J (1992) 0.83
Purification and immunological quantification of rat liver lysosomal glycosidases. Biochem J (1989) 0.80
Dog GM1 gangliosidosis: characterization of the residual liver acid beta-galactosidase. Am J Hum Genet (1980) 0.79
The binding of human liver acid beta-galactosidase to wheat-germ lectin is influenced by aggregation state of the enzyme. Biochem J (1982) 0.77
Protein measurement with the Folin phenol reagent. J Biol Chem (1951) 1743.91
The reliability of molecular weight determinations by dodecyl sulfate-polyacrylamide gel electrophoresis. J Biol Chem (1969) 142.11
CHROMATOGRAPHIC SEPARATION OF HUMAN BRAIN GANGLIOSIDES. J Neurochem (1963) 6.22
Estimation of molecular size and molecular weights of biological compounds by gel filtration. Methods Biochem Anal (1970) 4.48
The determination of carbohydrate in biological materials by gas-liquid chromatography. Methods Biochem Anal (1971) 3.20
Analytical fractionation of plant and animal proteins by gel electrofocusing. J Chromatogr (1968) 2.57
Generalized gangliosidosis: beta-galactosidase deficiency. Science (1968) 2.51
GM1 ganglioside beta-galactosidase. A. Purification and studies of the enzyme from human liver. J Biol Chem (1974) 1.58
The role of glycosidically bound mannose in the assimilation of beta-galactosidase by generalized gangliosidosis fibroblasts. Biochem Biophys Res Commun (1976) 1.53
The purification of papain by affinity chromatography. Eur J Biochem (1970) 1.52
Separation and isolation of rat and human intestinal beta-galactosidases. J Biol Chem (1969) 1.52
Purification and chemical properities of mouse liver lysosomal (L form) beta-glucuronidase. J Biol Chem (1975) 1.52
Binding of human liver beta-galactosidases to plant lectins insolubilized on agarose. Biochem Biophys Res Commun (1974) 1.40
The purification and characterization of rat liver lysosomal alpha-L-fucosidase. J Biol Chem (1977) 1.39
Beta-glucuronidase of rat preputial gland. Crystallization, properties, carbohydrate composition, and subunits. J Biochem (1975) 1.34
Ganglioside GM1 beta-galactosidase: studies in human liver and brain. Arch Biochem Biophys (1973) 1.31
Hydrolysis of GM1-ganglioside by human liver beta-galactosidase isoenzymes. Biochem J (1973) 1.25
The separation and characterization of the methylumbelliferyl beta-galactosidases of human liver. Biochem J (1976) 1.11
Purification of human liver acid beta-D-galactosidases using affinity chromatography. Anal Biochem (1976) 1.09
Carbohydrate composition of purified human liver alpha-L-fucosidase. Mol Cell Biochem (1977) 0.93
Effects of de-icing salt on ground water characteristics. Environ Lett (1975) 0.87
Multiple forms of glycosidases in the normal and pathological states. Enzyme (1974) 0.87
Purified human liver acid beta-D-galactosidases possessing activity towards G(M1)-ganglioside and lactosylceramide. Biochem J (1977) 0.81
Tay-Sachs disease: generalized absence of a beta-D-N-acetylhexosaminidase component. Science (1969) 5.77
Tay-sachs disease. Detection of heterozygotes and homozygotes by serum hexosaminidase assay. N Engl J Med (1970) 2.75
Cloning and expression of human arylsulfatase A. J Biol Chem (1989) 2.55
Gaucher's disease: deficiency of 'acid' -glucosidase and reconstitution of enzyme activity in vitro. Proc Natl Acad Sci U S A (1971) 2.53
Generalized gangliosidosis: beta-galactosidase deficiency. Science (1968) 2.51
Lipid composition of the normal human brain: gray matter, white matter, and myelin. J Lipid Res (1965) 2.35
Leaky gut in alcoholic cirrhosis: a possible mechanism for alcohol-induced liver damage. Am J Gastroenterol (1999) 2.22
Chromogenic immunodetection of human serum albumin and alpha-L-fucosidase clones in a human hepatoma cDNA expression library. DNA (1984) 2.15
Molecular cloning of a cDNA for human alpha-L-fucosidase. Proc Natl Acad Sci U S A (1985) 2.14
Direct sequencing from low-melt agarose with Sequenase. Nucleic Acids Res (1989) 2.09
I-cell disease: biochemical studies. Pediatr Res (1972) 2.08
Isolation and fatty acid composition of the plant sulfolipid and galactolipids. J Lipid Res (1964) 1.99
Sialidosis: a review of human neuraminidase deficiency. Am J Hum Genet (1979) 1.86
Point mutations in the DNA polymerase gene of human cytomegalovirus that result in resistance to antiviral agents. J Virol (1992) 1.81
Ganglioside storage diseases. Fed Proc (1971) 1.76
Adult Gaucher's disease: kindred studies and demonstration of a deficiency of acid beta-glucosidase in cultured fibroblasts. Am J Hum Genet (1972) 1.74
Exacerbation of alcoholic liver injury by enteral endotoxin in rats. Hepatology (2000) 1.73
Tay-Sachs disease: prenatal diagnosis. Science (1971) 1.65
Saposins: structure, function, distribution, and molecular genetics. J Lipid Res (1992) 1.64
Common variant in AMPD1 gene predicts improved clinical outcome in patients with heart failure. Circulation (1999) 1.62
Molecular basis of AMP deaminase deficiency in skeletal muscle. Proc Natl Acad Sci U S A (1992) 1.61
Synthesis of 2'-(4-methylumbelliferyl)-alpha-D-N-acetylneuraminic acid and detection of skin fibroblast neuraminidase in normal humans and in sialidosis. Biochemistry (1979) 1.60
GM1 ganglioside beta-galactosidase. A. Purification and studies of the enzyme from human liver. J Biol Chem (1974) 1.58
Differential effect of chloride ions on -galactosidase isoenzymes: a method for separate assay. Clin Chim Acta (1971) 1.56
Kinky hair disease. II. Biochemical studies. J Neuropathol Exp Neurol (1966) 1.55
Human glutamine phosphoribosylpyrophosphate amidotransferase. Kinetic and regulatory properties. J Biol Chem (1973) 1.53
Proposed changes for NIH's Center for Scientific Review. Panel on Scientific Boundaries for Review. Center for Scientific Review Advisory Committee, National Institutes of Health. Science (1999) 1.52
Preventing gut leakiness by oats supplementation ameliorates alcohol-induced liver damage in rats. J Pharmacol Exp Ther (2001) 1.46
Analysis of risk factors for the development of bronchiolitis obliterans syndrome. Am J Respir Crit Care Med (1999) 1.44
X-linked recessive nephrolithiasis: presentation and diagnosis in children. J Pediatr (1998) 1.44
The differing effects of acute and chronic alcohol on gastric and intestinal permeability. Am J Gastroenterol (1994) 1.41
Binding of human liver beta-galactosidases to plant lectins insolubilized on agarose. Biochem Biophys Res Commun (1974) 1.40
Characterization of unusual hexosaminidase A (HEX A) deficient human mutants. Am J Hum Genet (1978) 1.40
5-Amino-4-imidazolecarboxamide riboside (Z-riboside) metabolism in eukaryotic cells. J Biol Chem (1985) 1.40
Characterization of the human and rat myoadenylate deaminase genes. J Biol Chem (1990) 1.40
Fatty acid and fatty aldehyde composition of the major brain lipids in normal human gray matter, white matter, and myelin. J Lipid Res (1965) 1.39
Prolonged myocardial nucleotide depletion after brief ischemia in the open-chest dog. Am J Physiol (1982) 1.39
Sanfilippo syndrome: profound deficiency of alpha-acetylglucosaminidase activity in organs and skin fibroblasts from type-B patients. Proc Natl Acad Sci U S A (1972) 1.38
Absence of -N-acetyl-D-hexosaminidase A activity in a healthy woman. Am J Hum Genet (1973) 1.36
Saposin A: second cerebrosidase activator protein. Proc Natl Acad Sci U S A (1989) 1.35
Identification of a neurotrophic sequence in erythropoietin. Int J Mol Med (1998) 1.34
Human liver alpha-L-fucosidase. Purification, characterization, and immunochemical studies. J Biol Chem (1975) 1.34
Fucosidosis revisited: a review of 77 patients. Am J Med Genet (1991) 1.32
Determination of protein carbonyl groups by immunoblotting. Anal Biochem (1999) 1.32
Ganglioside GM1 beta-galactosidase: studies in human liver and brain. Arch Biochem Biophys (1973) 1.31
Ganglioside GM2 storage diseases: hexosaminidase deficiencies in cultured fibroblasts. Am J Hum Genet (1971) 1.29
Quantification and fatty acid and fatty aldehyde composition of ethanolamine, choline, and serine glycerophosphatides in human cerebral grey and white matter. J Lipid Res (1964) 1.27
The enzymatic synthesis of 5-amino-4-imidazolecarboxamide riboside triphosphate (ZTP). Science (1984) 1.26
Suggestions for a nomenclature for the GM2 gangliosidoses making certain (possibly unwarrantable) assumptions. Am J Hum Genet (1978) 1.24
Metabolism of 5-amino-4-imidazolecarboxamide riboside in cardiac and skeletal muscle. Effects on purine nucleotide synthesis. J Biol Chem (1982) 1.23
Juvenile GM2 gangliosidosis: partial deficiency of hexosaminidase A. J Pediatr (1970) 1.23
Hurler's syndrome: deficiency of a specific beta galactosidase isoenzyme. Science (1969) 1.23
Hyaluronidase and sulfatase deficiency in Hurler's syndrome. Biochem Med (1970) 1.23
Distribution of saposin proteins (sphingolipid activator proteins) in lysosomal storage and other diseases. Proc Natl Acad Sci U S A (1990) 1.22
Ganglioside GM2 N-acetyl-beta-D-galactosaminidase and asialo GM2 (GA2) N-acetyl-beta-D-galactosaminidase; studies in human skin fibroblasts. Clin Genet (1977) 1.21
Reversal of creatine kinase translational repression by 3' untranslated sequences. Science (1990) 1.21
Spectrum of mutations in fucosidosis. Eur J Hum Genet (1999) 1.19
Five gangliosidoses. Lancet (1969) 1.18
Studies on the exaggerated natriuretic response to a saline infusion in the hypothyroid rat. J Clin Invest (1970) 1.17
Glucocerebrosidase: reconstitution of activity from macromolecular components. Biochem J (1973) 1.17
Electrophoretic forms of human liver alpha-L-fucosidase and their relationship to fucosidosis (mucopolysaccharidosis F). Clin Chim Acta (1974) 1.16
Binding and transport of gangliosides by prosaposin. Proc Natl Acad Sci U S A (1992) 1.15
Aspiration of the asymptomatic metatarsophalangeal joint in gout patients and hyperuricemic controls. Arthritis Rheum (1982) 1.13
The fatty acid composition of brain sphingolipids: sphingomyelin, ceramide, cerebroside, and cerebroside sulfate. J Lipid Res (1964) 1.13
Nonuniform deficiency of hexosaminidase A in tissues and fluids of two unrelated individuals. Pediatr Res (1982) 1.13
Cerebral spongy degeneration of infancy. A biochemical and ultrastructural study of affected twins. Neurology (1972) 1.13
Isoenzyme patterns of human liver alpha-L-fucosidase during development. Dev Biol (1975) 1.13
Molecular genetics of GM1 beta-galactosidase. Clin Genet (1975) 1.13
Saposin D: a sphingomyelinase activator. Biochem Biophys Res Commun (1988) 1.12
Spondyloepiphyseal dysplasia, corneal clouding, normal intelligence and acid beta-galactosidase deficiency. Clin Genet (1976) 1.12
Ultrastructure of cultured fibroblasts in I-cell disease. Am J Dis Child (1971) 1.11
Characterization of a mutation in a family with saposin B deficiency: a glycosylation site defect. Proc Natl Acad Sci U S A (1990) 1.10
Lysosomal proteolysis of prosaposin, the precursor of saposins (sphingolipid activator proteins): its mechanism and inhibition by ganglioside. Arch Biochem Biophys (1997) 1.10
Prosaptide prevents paclitaxel neurotoxicity. Neurotoxicology (1998) 1.09
Purification of human liver acid beta-D-galactosidases using affinity chromatography. Anal Biochem (1976) 1.09
Juvenile GM2 gangliosidosis. Biochemical and ultrastructural studies on a new variant of Tay-Sachs disease. Arch Neurol (1971) 1.09
Prosaptide activates the MAPK pathway by a G-protein-dependent mechanism essential for enhanced sulfatide synthesis by Schwann cells. FASEB J (1998) 1.08
Myoadenylate deaminase deficiency. Functional and metabolic abnormalities associated with disruption of the purine nucleotide cycle. J Clin Invest (1984) 1.08
Separation of glycoprotein-derived oligosaccharides by thin-layer chromatography. Anal Biochem (1979) 1.08
Alternative splicing: a mechanism for phenotypic rescue of a common inherited defect. J Clin Invest (1993) 1.07
Neurologic involvement in Urbach-Wiethe's disease (lipoid proteinosis). A clinical, ultrastructural, and chemical study. Neurology (1971) 1.07
Sphingolipid hydrolases in brain tissue of patients with generalized gangliodosis. Biochim Biophys Acta (1970) 1.07
An electrophoretic variant of beta-galactosidase with altered catalytic properties in a patient with GM1 gangliosidosis. Proc Natl Acad Sci U S A (1975) 1.06
Segregation within a family of two mutant alleles for hexosaminidase A. Clin Genet (1976) 1.06
Cell death prevention, mitogen-activated protein kinase stimulation, and increased sulfatide concentrations in Schwann cells and oligodendrocytes by prosaposin and prosaptides. Proc Natl Acad Sci U S A (1997) 1.05
Intracellular transport of acid beta-glucosidase and lysosome-associated membrane proteins is affected in Gaucher's disease (G202R mutation). J Pathol (1999) 1.05
Genetic defects in glycoprotein metabolism. Annu Rev Genet (1983) 1.05
Mucolipidosis II and III: different residual activity of beta-galactosidase in cultured fibroblasts. Clin Genet (1976) 1.04
Human glutamine phosphoribosylpyrophosphate amidotransferase. Two molecular forms interconvertible by purine ribonucleotides and phosphoribosylpyrophosphate. J Biol Chem (1973) 1.04
Adenylate deaminase. A multigene family in humans and rats. J Biol Chem (1990) 1.04
Neuraminidase deficiency in the cherry red spot-myoclonus syndrome. Biochem Biophys Res Commun (1977) 1.04
Saposins (sap) A and C activate the degradation of galactosylceramide in living cells. FEBS Lett (1997) 1.03
Hypoxanthine phosphoribosyltransferase deficiency: association of reduced catalytic activity with reduced levels of immunologically detectable enzyme protein. Proc Natl Acad Sci U S A (1975) 1.03