Published in Clin Chim Acta on August 15, 1978
Tandem mass spectrometry for the direct assay of lysosomal enzymes in dried blood spots: application to screening newborns for mucopolysaccharidosis IVA. Clin Chem (2010) 1.33
Sanfilippo syndrome type C: deficiency of acetyl-CoA:alpha-glucosaminide N-acetyltransferase in skin fibroblasts. Proc Natl Acad Sci U S A (1978) 1.30
Review of clinical presentation and diagnosis of mucopolysaccharidosis IVA. Mol Genet Metab (2013) 1.17
Diagnosing mucopolysaccharidosis IVA. J Inherit Metab Dis (2013) 1.14
Current and emerging treatments and surgical interventions for Morquio A syndrome: a review. Res Rep Endocr Disord (2012) 1.08
Impaired degradation of keratan sulphate by Morquio A fibroblasts. Biochem J (1982) 0.96
Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta. Biochem J (1979) 0.95
Mucopolysaccharidosis IVA: identification of a common missense mutation I113F in the N-Acetylgalactosamine-6-sulfate sulfatase gene. Am J Hum Genet (1995) 0.89
Morquio syndrome (mucopolysaccharidosis IV B) associated with beta-galactosidase deficiency. Report of two cases. Am J Hum Genet (1980) 0.86
Enzyme replacement therapy for treating mucopolysaccharidosis type IVA (Morquio A syndrome): effect and limitations. Expert Opin Orphan Drugs (2015) 0.83
Mucopolysaccharidosis IVA: four new exonic mutations in patients with N-acetylgalactosamine-6-sulfate sulfatase deficiency. Am J Hum Genet (1996) 0.79
Mucopolysaccharidosis IVA and glycosaminoglycans. Mol Genet Metab (2016) 0.77
Quantitative aspects of pinocytosis and the intracellular fate of N-acetyl-alpha-D-glucosaminidase in Sanfilippo B fibroblasts. J Clin Invest (1974) 1.92
Biosynthesis of proteodermatan sulfate in cultured human fibroblasts. J Biol Chem (1984) 1.71
Generation of Arabidopsis thaliana plants with complex N-glycans lacking beta1,2-linked xylose and core alpha1,3-linked fucose. FEBS Lett (2004) 1.70
Interaction of small dermatan sulfate proteoglycan from fibroblasts with fibronectin. J Cell Biol (1987) 1.68
Interaction of biglycan with type I collagen. J Biol Chem (1995) 1.63
Decorin-type I collagen interaction. Presence of separate core protein-binding domains. J Biol Chem (1995) 1.45
Small proteoglycans in human diabetic nephropathy: discrepancy between glomerular expression and protein accumulation of decorin, biglycan, lumican, and fibromodulin. FASEB J (2001) 1.45
The Sanfilippo A corrective factor. Purification and mode of action. J Biol Chem (1972) 1.41
Sanfilippo A disease in the fetus. J Med Genet (1974) 1.40
Cloning and expression of a proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I. A seventh member of the human beta4-galactosyltransferase gene family. J Biol Chem (1999) 1.37
Identification and characterization of (GA/CT)n-microsatellite loci from Quercus petraea. Plant Mol Biol (1997) 1.35
A genetic defect in the biosynthesis of dermatan sulfate proteoglycan: galactosyltransferase I deficiency in fibroblasts from a patient with a progeroid syndrome. Proc Natl Acad Sci U S A (1990) 1.34
Metabolism of sulfated glycosaminoglycans in cultured endothelial cells and smooth muscle cells from bovine aorta. Biochim Biophys Acta (1978) 1.33
Sanfilippo disease type D: deficiency of N-acetylglucosamine-6-sulfate sulfatase required for heparan sulfate degradation. Proc Natl Acad Sci U S A (1980) 1.33
Insect cells contain an unusual, membrane-bound beta-N-acetylglucosaminidase probably involved in the processing of protein N-glycans. J Biol Chem (1995) 1.31
Sanfilippo syndrome type C: deficiency of acetyl-CoA:alpha-glucosaminide N-acetyltransferase in skin fibroblasts. Proc Natl Acad Sci U S A (1978) 1.30
Maturation of human procathepsin B. Proenzyme activation and proteolytic processing of the precursor to the mature proteinase, in vitro, are primarily unimolecular processes. J Biol Chem (1994) 1.29
Identification of microsatellite sequences in Vitis riparia and their applicability for genotyping of different Vitis species. Genome (1999) 1.27
Processing of asparagine-linked oligosaccharides in insect cells. N-acetylglucosaminyltransferase I and II activities in cultured lepidopteran cells. Glycobiology (1993) 1.25
The sanfilippo B corrective factor: a N-acetyl-alpha-D-glucosamindiase. Biochem Biophys Res Commun (1972) 1.25
Liberation of N-acetylglucosamine-6-sulfate by human beta-N-acetylhexosaminidase A. J Biol Chem (1981) 1.23
Metabolism of sulfated glycosaminoglycans in cultivated bovine arterial cells. I. Characterization of different pools of sulfated glycosaminoglycans. Hoppe Seylers Z Physiol Chem (1975) 1.23
Molecular cloning and functional expression of beta1, 2-xylosyltransferase cDNA from Arabidopsis thaliana. FEBS Lett (2000) 1.21
Biosynthesis of cathepsin B in cultured normal and I-cell fibroblasts. J Biol Chem (1987) 1.19
Generation of co-dominant PCR-based markers by duplex analysis on high resolution gels. Plant J (1998) 1.18
Influence of decorin on fibroblast adhesion to fibronectin. Eur J Cell Biol (1991) 1.16
Glycosaminoglycan-free small proteoglycan core protein is secreted by fibroblasts from a patient with a syndrome resembling progeroid. Am J Hum Genet (1987) 1.15
Decorin-mediated signal transduction in endothelial cells. Involvement of Akt/protein kinase B in up-regulation of p21(WAF1/CIP1) but not p27(KIP1). J Biol Chem (2001) 1.15
Interactions between thrombospondin and the small proteoglycan decorin: interference with cell attachment. Eur J Cell Biol (1992) 1.13
N-Acetylglucosamine-6-sulfate sulfatase from human urine. J Biol Chem (1979) 1.13
Biochemical heterogeneity of the Sanfilippo syndrome: preliminary characterization of two deficient factors. Biochem Biophys Res Commun (1971) 1.12
Mucopolysaccharidosis 3 A (Sanfilippo A disease): deficiency of a heparin sulfamidase in skin fibroblasts and leucocytes. Biochem Biophys Res Commun (1973) 1.12
Activation of procathepsin B in human hepatoma cells: the conversion into the mature enzyme relies on the action of cathepsin B itself. Biochem J (1993) 1.12
Immunocytochemical investigation on the distribution of small chondroitin sulfate-dermatan sulfate proteoglycan in the human. J Histochem Cytochem (1986) 1.11
Selective inactivity of TGF-beta/decorin complexes. FEBS Lett (1994) 1.10
Influence of chlorate on proteoglycan biosynthesis by cultured human fibroblasts. J Biol Chem (1988) 1.10
Mucopolysaccharidosis type II (Hunter syndrome): mutation "hot spots" in the iduronate-2-sulfatase gene. Am J Hum Genet (1996) 1.09
Receptor-mediated endocytosis of proteoglycans by human fibroblasts involves recognition of the protein core. Biochem J (1983) 1.09
Decorin core protein fragment Leu155-Val260 interacts with TGF-beta but does not compete for decorin binding to type I collagen. Arch Biochem Biophys (1998) 1.09
Comparison of small proteoglycans from skin fibroblasts and vascular smooth-muscle cells. Biochem J (1986) 1.08
Metabolism of sulfated glycosaminoglycans in cultivated bovine arterial cells. II. Quantitative studies on the uptake of 35SO4-labeled proteoglycans. Hoppe Seylers Z Physiol Chem (1975) 1.07
Molecular cloning and characterization of cDNA coding for beta1, 2N-acetylglucosaminyltransferase I (GlcNAc-TI) from Nicotiana tabacum. Glycobiology (1999) 1.06
Heterogeneity of Morquio disease. Clin Genet (1986) 1.05
Light and electron microscopical immunohistochemical localization of the small proteoglycan core proteins decorin and biglycan in human knee joint cartilage. Histochem J (1994) 1.03
Tay-Sachs disease: one-step assay of beta-N-acetylhexosaminidase in serum with a sulphated chromogenic substrate. Clin Chim Acta (1983) 1.03
Modulation of invasive properties of murine squamous carcinoma cells by heterologous expression of cathepsin B and cystatin C. Int J Cancer (1999) 1.01
Endocytosis of a small dermatan sulphate proteoglycan. Identification of binding proteins. Biochem J (1989) 1.01
Purification, cDNA cloning, and expression of GDP-L-Fuc:Asn-linked GlcNAc alpha1,3-fucosyltransferase from mung beans. J Biol Chem (1999) 1.01
Molecular cloning of cDNA encoding N-acetylglucosaminyltransferase II from Arabidopsis thaliana. Glycoconj J (1999) 0.99
Beta-glucuronidase deficiency in a girl with unusual clinical features. Eur J Pediatr (1977) 0.99
Influence of decorin expression on transforming growth factor-beta-mediated collagen gel retraction and biglycan induction. Matrix Biol (2000) 0.99
Immunohistochemical localization of the small proteoglycans decorin and biglycan in human intervertebral discs. Cell Tissue Res (1997) 0.99
Small proteoglycans of normal adult human kidney: distinct expression patterns of decorin, biglycan, fibromodulin, and lumican. Kidney Int (2000) 0.98
Paracrine or virus-mediated induction of decorin expression by endothelial cells contributes to tube formation and prevention of apoptosis in collagen lattices. Eur J Cell Biol (1999) 0.98
Antisense inhibition of decorin expression in myoblasts decreases cell responsiveness to transforming growth factor beta and accelerates skeletal muscle differentiation. J Biol Chem (2000) 0.98
Uptake of proteoglycans and sulfated glycosaminoglycans by cultured skin fibroblasts. Eur J Biochem (1978) 0.97
Presence of small proteoglycan fragments in normal and arthritic human cartilage. Arthritis Rheum (1992) 0.97
Removal of 106 amino acids from the N-terminus of UDP-GlcNAc: alpha-3-D-mannoside beta-1,2-N-acetylglucosaminyltransferase I does not inactivate the enzyme. Glycoconj J (1998) 0.97
Endocytosis of different members of the small chondroitin/dermatan sulfate proteoglycan family. J Biol Chem (1992) 0.96
Differential expression of the small chondroitin/dermatan sulfate proteoglycans decorin and biglycan after injury of the adult rat brain. Brain Res (1995) 0.96
Impaired degradation of keratan sulphate by Morquio A fibroblasts. Biochem J (1982) 0.96
Sandhoff disease: defective glycosaminoglycan catabolism in cultured fibroblasts and its correction by beta-N-acetylhexosaminidase. Eur J Biochem (1974) 0.95
Noncovalent complexes between the lysosomal proteinase cathepsin B and its propeptide account for stable, extracellular, high molecular mass forms of the enzyme. J Biol Chem (1994) 0.95
The N-terminal 77 amino acids from tobacco N-acetylglucosaminyltransferase I are sufficient to retain a reporter protein in the Golgi apparatus of Nicotiana benthamiana cells. FEBS Lett (1999) 0.95
Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta. Biochem J (1979) 0.95
Interaction of the small proteoglycan decorin with fibronectin. Involvement of the sequence NKISK of the core protein. Biochem J (1991) 0.95
Uptake of hyaluronate by cultured cells. Biochem Biophys Res Commun (1977) 0.94
Decorin, biglycan and their endocytosis receptor in rat renal cortex. Kidney Int (1998) 0.94
Stachyose synthesis in seeds of adzuki bean (Vigna angularis): molecular cloning and functional expression of stachyose synthase. Plant J (1999) 0.93
Evidence for degradation of heparan sulfate by endoglycosidases: glucosamine and hexuronic acid are reducing terminals of intracellular heparan sulfate from human skin fibroblasts. Biochem Biophys Res Commun (1976) 0.93
Peripheral distribution of dermatan sulfate proteoglycans (decorin) in amyloid-containing plaques and their presence in neurofibrillary tangles of Alzheimer's disease. J Histochem Cytochem (1992) 0.93
Experimental evidence for an achiral orthogonal biaxial smectic phase without in-plane order exhibiting antiferroelectric switching behavior. Phys Rev E Stat Nonlin Soft Matter Phys (2001) 0.93
Expression of biglycan, decorin and proteoglycan-100/CSF-1 in normal and fibrotic human liver. Pathol Res Pract (1997) 0.92
Enzymic diagnosis of the genetic mucopolysaccharide storage disorders. Methods Enzymol (1982) 0.91
Non-uniform influence of transforming growth factor-beta on the biosynthesis of different forms of small chondroitin sulphate/dermatan sulphate proteoglycan. Biochem J (1990) 0.91
Clinical heterogeneity in Sanfilippo disease (mucopolysaccharidosis III) type D: presentation of two new cases. Eur J Pediatr (1983) 0.90
Purification and partial characterization of a novel lectin from elder (Sambucus nigra L.) fruit. Biochem J (1991) 0.90
Matrix metalloproteinase expression by endothelial cells in collagen lattices changes during co-culture with fibroblasts and upon induction of decorin expression. J Cell Physiol (2001) 0.90
Differences in decorin expression by papillary and reticular fibroblasts in vivo and in vitro. Biochem J (1993) 0.90
[Macromolecular polysaccharide proteins. 3. Metabolic heterogeneity of chondroitin-4-sulfate proteins and collagen metabolism in bovine nasal cartilage]. Hoppe Seylers Z Physiol Chem (1968) 0.90
Sanfilippo type C disease: clinical findings in four patients with a new variant of mucopolysaccharidosis III. Eur J Pediatr (1979) 0.89
The small proteoglycans decorin and biglycan in human articular cartilage of late-stage osteoarthritis. Osteoarthritis Cartilage (2001) 0.89
Localization of sulfated glycosaminoglycans within cell nuclei by high-resolution autoradiography. Exp Cell Res (1976) 0.89
Post-translational phosphorylation of proteodermatan sulfate. J Biol Chem (1986) 0.89
Dyggve-Melchior-Clausen syndrome: normal degradation of proteodermatan sulfate, proteokeratan sulfate and heparan sulfate. Clin Chim Acta (1984) 0.89