| heparan N- |
a lysosomal enzyme of the hydrolase class that catalyzes the removal of sulfate from terminal N-sulfated glucosamine residues of heparan sulfate chains. Deficiency of the enzyme, an autosomal recessive trait, causes Sanfilippo's syndrome, type A. See also N-sulfoglucosamine sulfohydrolase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| heparan sulfate |
a glycosaminoglycan occurring in the cell membrane of most cells. It consists of repeating disaccharide units in specific linkage, each composed of a glucosamine residue linked to a uronic acid, either glucuronic acid or L-iduronic acid, which may be sulfated. The glucosamine residues frequently contain acetyl or sulfate group substituents. It is an accumulation product in several mucopolysaccharidoses.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| heparan sulfate sulfamidase |
heparan N-sulfatase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| heparan-α-glucosaminide N- |
[EC 2.3.1.78] a lysosomal enzyme of the transferase class that catalyzes the acetylation of the amino groups of terminal desulfated glucosamine residues in heparan sulfate chains, a step in the degradation of heparan sulfate. Acetyl coenzyme A is the acetyl donor. Deficiency of the enzyme, an autosomal recessive trait, results in Sanfilippo's syndrome, type C. Called also acetyl CoA:α-glucosaminide N-acetyltransferase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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