Published in Biochem J on July 01, 1973
The separation and characterization of bronchial glycoproteins by density-gradient methods. Biochem J (1977) 1.86
The effect of D-xylose, beta-D-xylosides and beta-D-galactosides on chondroitin sulphate biosynthesis in embryonic chicken cartilage. Biochem J (1975) 1.70
The molecular-weight distribution of glycosaminoglycans. Biochem J (1973) 1.48
The alkali-labile linkage between keratan sulphate and protein. Biochem J (1974) 1.27
Control of proteoglycan synthesis. Studies on the activation of synthesis observed during culture of articular cartilages. Biochem J (1980) 1.24
Constant and variable domains of different disaccharide structure in corneal keratan sulphate chains. Biochem J (1987) 1.19
Proteoglycans of the intervertebral disc. Homology of structure with laryngeal proteoglycans. Biochem J (1979) 1.15
The structure and composition of cartilage keratan sulphate. Biochem J (1974) 1.04
Studies on the polydispersity and heterogeneity of cartilage proteoglycans. Identification of 3 proteoglycan structures in bovine nasal cartilage. Biochem J (1975) 1.01
Macromolecular distribution near the limits of density-gradient columns. Some applications to the separation and fractionation of glycoproteins. Biochem J (1977) 0.98
Effect of cycloheximide, beta-D-xylosides and beta-D-galactosides on heparin biosynthesis in mouse mastocytoma. Biochem J (1981) 0.90
The synthesis of hyaluronic acid by sheep and rabbit articular cartilage in vitro. Biochem J (1975) 0.89
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Proteinpolysaccharide complex from bovine nasal cartilage. The function of glycoprotein in the formation of aggregates. J Biol Chem (1969) 6.87
Proteinpolysaccharide complex from bovine nasal cartilage. A comparison of low and high shear extraction procedures. J Biol Chem (1969) 4.65
The nature of the link between protein and carbohydrate of a chondroitin sulphate complex from hyaline cartilage. Biochem J (1958) 4.62
The simultaneous estimation of 6-deoxy-L-galactose (L-fucose), D-mannose, D-galactose, 2-acetamido-2-deoxy-D-glucose (N-acetyl-D-glucosamine) and N-acetylneuraminic acid (sialic acid) in glycopeptides and glycoproteins. Biochim Biophys Acta (1967) 2.95
THE O-SERINE LINKAGE IN PEPTIDES OF CHONDROITIN 4- OR 6-SULFATE. J Biol Chem (1965) 2.60
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The structure of a glycopeptide isolated from the yeast cell wall. Biochem J (1968) 2.05
THE ROLE OF GALACTOSE AND XYLOSE IN THE LINKAGE OF HEPARIN TO PROTEIN. J Biol Chem (1965) 1.96
Studies on protein-polysaccharides from pig laryngeal cartilage. Heterogeneity, fractionation and characterization. Biochem J (1969) 1.94
The separation of new forms of the proteinpolysaccharides of bovine nasal cartilage. J Biol Chem (1966) 1.61
Immunochemical studies on blood groups. XXXIV. Structures of some oligosaccharides produced by alkaline degradation of blood group A, B, and H substances. Biochemistry (1966) 1.45
The sulfation of chondroitin sulfate in embryonic chick cartilage epiphyses. J Biol Chem (1969) 1.43
The determination of molecular weights of biological macromolecules by ultracentrifuge methods. Prog Biophys Mol Biol (1967) 1.42
The composition and physicochemical properties of bovine nasal-septa protein-polysaccharide complex. Biochem J (1967) 1.38
The Lobry de Bruyn-Alberda van Ekenstein transformation. Adv Carbohydr Chem (1958) 0.92
Physical characterization of dermatan sulphate-protein. Arch Biochem Biophys (1968) 0.91
Studies on porcine costal cartilage protein-polysaccharide complex. II. Chemical studies. Biochim Biophys Acta (1966) 0.83
[Study on glycoproteins. Demonstration of the O-seryl and O-threonyl-N-acetylgalactosaminidic bonds in the gamma A globulins of human milk]. C R Acad Sci Hebd Seances Acad Sci D (1968) 0.80
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Prevalence of lysosomal storage disorders. JAMA (1999) 9.06
The physician's actions and the outcome of illness in family practice. J Fam Pract (1986) 4.41
Huntington disease-linked locus D4S111 exposed as the alpha-L-iduronidase gene. Somat Cell Mol Genet (1991) 2.23
A proteoglycan form of heparin and its degradation to single-chain molecules. J Biol Chem (1978) 2.18
The biosynthesis of chondroitin-sulfate protein complex. Proc Natl Acad Sci U S A (1965) 2.17
Chromosomal localization of ARSB, the gene for human N-acetylgalactosamine-4-sulphatase. Hum Genet (1989) 1.95
Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA. Proc Natl Acad Sci U S A (1990) 1.91
Glycosaminoglycan synthesis by cultured human skin fibroblasts after transformation with simian virus 40. J Biol Chem (1977) 1.77
The effect of serum on biosynthesis of proteoglycans by bovine articular cartilage in culture. Arch Biochem Biophys (1983) 1.71
The effect of D-xylose, beta-D-xylosides and beta-D-galactosides on chondroitin sulphate biosynthesis in embryonic chicken cartilage. Biochem J (1975) 1.70
A mouse model for mucopolysaccharidosis type III A (Sanfilippo syndrome). Glycobiology (1999) 1.67
Diagnosis of Maroteaux-Lamy syndrome by the use of radiolabelled oligosaccharides as substrates for the determination of arylsulphatase B activity. Biochem J (1986) 1.61
Genetic mapping of new RFLPs at Xq27-q28. Genomics (1991) 1.60
The biosynthesis of chondroitin sulfate. Arch Biochem Biophys (1966) 1.58
Structure of a human lysosomal sulfatase. Structure (1997) 1.57
Molecular genetics of mucopolysaccharidosis type I: diagnostic, clinical, and biological implications. Hum Mutat (1995) 1.54
Mechanism of catabolism of aggrecan by articular cartilage. Arch Biochem Biophys (1992) 1.54
Molecular genetics of mucopolysaccharidosis type IIIA and IIIB: Diagnostic, clinical, and biological implications. Hum Mutat (2001) 1.52
Human liver iduronate-2-sulphatase. Purification, characterization and catalytic properties. Biochem J (1990) 1.50
Human alpha-L-iduronidase: cDNA isolation and expression. Proc Natl Acad Sci U S A (1991) 1.48
The molecular-weight distribution of glycosaminoglycans. Biochem J (1973) 1.48
alpha-L-iduronidase, beta-D-glucuronidase, and 2-sulfo-L-iduronate 2-sulfatase: preparation and characterization of radioactive substrates from heparin. Carbohydr Res (1979) 1.45
Atypical myoglobinuria. Vet Rec (1991) 1.44
High-resolution electrophoresis of urinary glycosaminoglycans: an improved screening test for the mucopolysaccharidoses. Anal Biochem (1982) 1.43
The sulfation of chondroitin sulfate in embryonic chick cartilage epiphyses. J Biol Chem (1969) 1.43
Enzymatic diagnosis of the mucopolysaccharidoses: experience of 96 cases diagnosed in a five-year period. Med J Aust (1982) 1.41
Coexistence of Gaucher disease type 1 and Joubert syndrome. J Med Genet (1998) 1.40
Enzyme replacement therapy for mucopolysaccharidosis VI from 8 weeks of age--a sibling control study. Clin Genet (2009) 1.39
Human alpha-L-iduronidase. 1. Purification, monoclonal antibody production, native and subunit molecular mass. Eur J Biochem (1985) 1.39
Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation. Hum Mol Genet (2001) 1.36
Human N-acetylgalactosamine-4-sulphate sulphatase. Purification, monoclonal antibody production and native and subunit Mr values. Biochem J (1987) 1.34
Human liver sulphamate sulphohydrolase. Determinations of native protein and subunit Mr values and influence of substrate agylcone structure on catalytic properties. Biochem J (1986) 1.33
Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): six unique arylsulfatase B gene alleles causing variable disease phenotypes. Am J Hum Genet (1994) 1.33
Correction of human mucopolysaccharidosis type-VI fibroblasts with recombinant N-acetylgalactosamine-4-sulphatase. Biochem J (1992) 1.32
Structure and sequence of the human alpha-L-iduronidase gene. Genomics (1992) 1.32
A strategy for rapid sequencing of heparan sulfate and heparin saccharides. Proc Natl Acad Sci U S A (1999) 1.27
A fluorometric assay using 4-methylumbelliferyl alpha-L-iduronide for the estimation of alpha-L-iduronidase activity and the detection of Hurler and Scheie syndromes. Clin Chim Acta (1979) 1.27
Highly sensitive sequencing of the sulfated domains of heparan sulfate. J Biol Chem (1999) 1.27
The alkali-labile linkage between keratan sulphate and protein. Biochem J (1974) 1.27
Human alpha-L-iduronidase. 2. Catalytic properties. Eur J Biochem (1985) 1.26
Enzyme replacement therapy in a feline model of Maroteaux-Lamy syndrome. J Clin Invest (1996) 1.24
Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies. Biochem J (1989) 1.24
Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B). Hum Mol Genet (1996) 1.22
The diagnosis of the Sanfilippo C syndrome, using monosaccharide and oligosaccharide substrates to assay acetyl-CoA: 2-amino-2-deoxy-alpha-glucoside N-acetyltransferase activity. Clin Chim Acta (1981) 1.22
Determination of acid alpha-glucosidase activity in blood spots as a diagnostic test for Pompe disease. Clin Chem (2001) 1.22
Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization. Biochem J (1987) 1.21
Pharmacokinetic profile of recombinant human N-acetylgalactosamine 4-sulphatase enzyme replacement therapy in patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): a phase I/II study. Acta Paediatr Suppl (2005) 1.18
Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes. Am J Hum Genet (1993) 1.18
Mucopolysaccharidosis type I: identification of 8 novel mutations and determination of the frequency of the two common alpha-L-iduronidase mutations (W402X and Q70X) among European patients. Hum Mol Genet (1994) 1.18
Sanfilippo type B syndrome (mucopolysaccharidosis III B): allelic heterogeneity corresponds to the wide spectrum of clinical phenotypes. Eur J Hum Genet (1999) 1.17
Recombinant human iduronate-2-sulphatase: correction of mucopolysaccharidosis-type II fibroblasts and characterization of the purified enzyme. Biochem J (1993) 1.17
Diagnosis of Sanfilippo type A syndrome by estimation of sulfamidase activity using a radiolabelled tetrasaccharide substrate. Clin Chim Acta (1982) 1.17
A common mutation for mucopolysaccharidosis type I associated with a severe Hurler syndrome phenotype. Hum Mutat (1992) 1.17
Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties. Biochem J (1987) 1.16
Frequent deletions at Xq28 indicate genetic heterogeneity in Hunter syndrome. Hum Genet (1991) 1.15
Biochemical discrimination of Hurler and Scheie syndromes. Clin Sci (Lond) (1979) 1.13
Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome. Nat Genet (1995) 1.13
alpha-L-iduronidase mutations (Q70X and P533R) associate with a severe Hurler phenotype. Hum Mutat (1992) 1.12
Enzyme replacement therapy in a feline model of MPS VI: modification of enzyme structure and dose frequency. Pediatr Res (2000) 1.12
alpha-L-Iduronidase deficiency in mucopolysaccharidosis type I against a radiolabelled sulfated disaccharide substrate derived from dermatan sulfate. Clin Genet (1984) 1.10
Defective lysosomal egress of free sialic acid (N-acetylneuraminic acid) in fibroblasts of patients with infantile free sialic acid storage disease. J Biol Chem (1989) 1.10
Identification and molecular characterization of alpha-L-iduronidase mutations present in mucopolysaccharidosis type I patients undergoing enzyme replacement therapy. Hum Mutat (2004) 1.09
Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase. Biochem Biophys Res Commun (1988) 1.09
Human N-acetylgalactosamine-4-sulphatase biosynthesis and maturation in normal, Maroteaux-Lamy and multiple-sulphatase-deficient fibroblasts. Biochem J (1990) 1.09
Enzymatic depolymerization of heparin-related polysaccharides. Substrate specificities of mouse mastocytoma and human platelet endo-beta-D-glucuronidases. J Biol Chem (1982) 1.08
The relation of protein synthesis to chondroitin sulphate biosynthesis in cultured bovine cartilage. Biochem J (1984) 1.08
Enzyme replacement therapy from birth in a feline model of mucopolysaccharidosis type VI. J Clin Invest (1997) 1.08
Detection of Morquio A syndrome using radiolabelled substrates derived from keratan sulphate for the estimation of galactose 6-sulphate sulphatase. Clin Sci (Lond) (1983) 1.07
A cDNA clone for human glucosamine-6-sulphatase reveals differences between arylsulphatases and non-arylsulphatases. Biochem J (1992) 1.07
Chromosomal localization of the human alpha-L-iduronidase gene (IDUA) to 4p16.3. Am J Hum Genet (1990) 1.05
The structure and composition of cartilage keratan sulphate. Biochem J (1974) 1.04
Genotype-phenotype correlations in mucopolysaccharidosis type I using enzyme kinetics, immunoquantification and in vitro turnover studies. Biochim Biophys Acta (1998) 1.04
Effect of high dose, repeated intra-cerebrospinal fluid injection of sulphamidase on neuropathology in mucopolysaccharidosis type IIIA mice. Genes Brain Behav (2008) 1.04
Glycosaminoglycan accumulation and excretion in the mucopolysaccharidoses: characterization and basis of a diagnostic test for MPS. Mol Genet Metab (1998) 1.03
A novel missense mutation in lysosomal sulfamidase is the basis of MPS III A in a spontaneous mouse mutant. Glycobiology (2001) 1.02
Characterization of a deletion at Xq27-q28 associated with unbalanced inactivation of the nonmutant X chromosome. Am J Hum Genet (1992) 1.02
Cleavage of proteoglycan aggregate by leucocyte elastase. Arch Biochem Biophys (1992) 1.02
Analysis of N-acetylgalactosamine-4-sulfatase protein and kinetics in mucopolysaccharidosis type VI patients. Am J Hum Genet (1991) 1.01
Studies on the polydispersity and heterogeneity of cartilage proteoglycans. Identification of 3 proteoglycan structures in bovine nasal cartilage. Biochem J (1975) 1.01
Iduronate-2-sulfatase gene mutations in 16 patients with mucopolysaccharidosis type II (Hunter syndrome). Hum Mol Genet (1993) 1.00
Identification, expression, and biochemical characterization of N-acetylgalactosamine-4-sulfatase mutations and relationship with clinical phenotype in MPS-VI patients. Am J Hum Genet (1996) 1.00
The isolation of S-sulphoglutathione from the small intestine of the rat. Biochem J (1964) 1.00
N-acetylglucosamine 6-sulfate residues in keratan sulfate and heparan sulfate are desulfated by the same enzyme. Biochem Int (1983) 1.00
Feline mucopolysaccharidosis type VI. Characterization of recombinant N-acetylgalactosamine 4-sulfatase and identification of a mutation causing the disease. J Biol Chem (1996) 0.99
Isolation and characterisation of a recombinant, precursor form of lysosomal acid alpha-glucosidase. Eur J Biochem (1995) 0.99
The sulphation of chondroitin sulphate in embryonic chicken cartilage. Biochem J (1969) 0.99
Juvenile form of mucopolysaccharidosis VI (Maroteaux-Lamy syndrome). A C-terminal extension causes instability but increases catalytic efficiency of arylsulfatase B. J Biol Chem (1994) 0.99
Human liver glucuronate 2-sulphatase. Purification, characterization and catalytic properties. Biochem J (1989) 0.99
Improved concanavalin A-Sepharose elution by specific readsorption of glycoproteins. J Chromatogr (1983) 0.99
Studies on the use of brilliant Green and a modified Endo's medium in the Isolation of Bacillus Typhosus from Feces. J Med Res (1916) 0.98
The extracellular processing and catabolism of hyaluronan in cultured adult articular cartilage explants. Arch Biochem Biophys (1992) 0.98
alpha-L-iduronidase in normal and mucopolysaccharidosis-type-I human skin fibroblasts. Biochem J (1991) 0.98
Conditional tissue-specific expression of the acid alpha-glucosidase (GAA) gene in the GAA knockout mice: implications for therapy. Hum Mol Genet (2001) 0.98
Radiolabelled oligosaccharides as substrates for the estimation of sulfamidase and the detection of the Sanfilippo type A syndrome. Clin Chim Acta (1981) 0.98
Sequence of the human iduronate 2-sulfatase (IDS) gene. Genomics (1993) 0.97