N-acetylglucosamine-6-sulfatase

N-acetylglucosamine-6-sulfatase also known as glucosamine (N-acetyl)-6-sulfatase is an is_associated_with::enzyme that in humans is encoded by the GNS is_associated_with::gene. This enzyme is deficient in is_associated_with::Sanfilippo Syndrome type IIId. This enzyme catalyses the following is_associated_with::chemical reaction:


 * is_associated_with::Hydrolysis of the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of is_associated_with::heparan sulfate and is_associated_with::keratan sulfate

Function
N-acetylglucosamine-6-sulfatase is a is_associated_with::lysosomal enzyme found in all cells. It is involved in the is_associated_with::catabolism of is_associated_with::heparin, is_associated_with::heparan sulphate, and is_associated_with::keratan sulphate.

Clinical significance
Deficiency of this enzyme results in the accumulation of undergraded substrate and the is_associated_with::lysosomal storage disorder is_associated_with::mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of is_associated_with::Sanfilippo syndrome.

Nomenclature
The systematic name of this enzyme is "N-acetyl-D-glucosamine-6-sulfate 6-sulfohydrolase". Other accepted names include:
 * N-acetylglucosamine-6-sulfatase,
 * glucosamine (N-acetyl)-6-sulfatase,
 * 2-acetamido-2-deoxy-D-glucose 6-sulfate sulfatase,
 * N-acetylglucosamine 6-sulfate sulfatase,
 * O,N-disulfate O-sulfohydrolase,
 * acetylglucosamine 6-sulfatase,
 * chondroitinsulfatase, and
 * glucosamine-6-sulfatase.