EC Number |
Natural Substrates |
---|
3.1.6.4 | more |
enzyme is involved in lysosomal degradation of the glycosaminoglycans keratan sulfate and chondroitin 6-sulfate |
3.1.6.4 | more |
enzyme is involved in chondroitin sulfate breakdown |
3.1.6.4 | more |
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 |
3.1.6.4 | more |
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 |
3.1.6.4 | more |
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 |