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Results 1 - 5 of 5
EC Number
Natural Substrates
Commentary (Nat. Sub.)
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enzyme is involved in lysosomal degradation of the glycosaminoglycans keratan sulfate and chondroitin 6-sulfate
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enzyme is involved in chondroitin sulfate breakdown
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concerted action of exoglycosidases and EC 3.1.6.4 in degradation of polymeric chondroitin 4-sulfate, no contribution of the enzyme to the degradation of chondroitin
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the enzyme catalyzes the first step of intralysosomal keratan sulfate catabolism. In Morquito A syndrome the N-acetylglucosamine 6-sulfate sulfatase deficiency causes the accumulation of keratan sulfate in tissue and results in generalized skeletal dysplasia in affected patients
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enzyme deficiency causes mucopolysaccharidosis type IV, i.e. Morquio A syndrome, an autosomal recessive inborn error, due to accumulation of sulfated glycosaminoglycans in lysosomes, overview
Results 1 - 5 of 5