Iduronate-2-sulfatase
Iduronate 2-sulfatase (EC 3.1.6.13; systematic name L-iduronate-2-sulfate 2-sulfohydrolase) is a sulfatase enzyme associated ... Iduronate-2-sulfatase has a strong sequence homology with human arylsulfatases A, B, and C, and human glucosamine-6-sulfatase. ... Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X- ... Li P, Huffman P, Thompson JN (1995). "Mutations of the iduronate-2-sulfatase gene on a T146T background in three patients with ...
Hunter syndrome
The IDS gene encodes for an enzyme called iduronate-2-sulfatase (I2S). A lack of this enzyme leads to a buildup of GAGs, which ... Hunter syndrome is caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase (I2S). The lack of this enzyme causes ... The first step in the breakdown of dermatan sulfate and heparan sulfate requires the lysosomal enzyme iduronate-2-sulfatase, or ...
Sulfatase
... isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA". Proc. Natl. Acad. Sci. U.S.A. 87 (21): 8531-8535 ... Many sulfatases are localized in the lysosome, an acidic digestive organelle found within the cell. Lysosomal sulfatases cleave ... Genetic defects in sulfatase activity can arise through mutations in individual sulfatases and result in certain lysosomal ... The following sulfatases have been shown to be structurally related based on their sequence homology: cerebroside-sulfatase ...
Mucopolysaccharidosis
MPS II, Hunter syndrome or iduronate sulfatase deficiency, is caused by lack of the enzyme iduronate sulfatase. Hunter syndrome ... Sanfilippo A is the most severe of the MPS III disorders and is caused by the missing or altered enzyme heparan N-sulfatase. ... Its two subtypes result from the missing or deficient enzymes N-acetylgalactosamine-6-sulfatase (GALNS) (Type A) or beta- ... Sanfilippo D is caused by the missing or deficient enzyme N-acetylglucosamine 6-sulfatase. The incidence of Sanfilippo syndrome ...
CXorf40A
"130 kb of DNA sequence reveals two new genes and a regional duplication distal to the human iduronate-2-sulfate sulfatase locus ...
Idursulfase
It is a purified form of the lysosomal enzyme iduronate-2-sulfatase and is produced by recombinant DNA technology in a human ...
Arylsulfatase B
Bhattacharyya S, Tobacman JK (2007). "Steroid sulfatase, arylsulfatases A and B, galactose-6-sulfatase, and iduronate sulfatase ... Arylsulfatase B (N-acetylgalactosamine-4-sulfatase, chondroitinsulfatase, chondroitinase, acetylgalactosamine 4-sulfatase, N- ... Aryl Sulfatase GRCh38: Ensembl release 89: ENSG00000113273 - Ensembl, May 2017 GRCm38: Ensembl release 89: ENSMUSG00000042082 ... Bhattacharyya S, Feferman L, Tobacman JK (2016). "Inhibition of Phosphatase Activity Follows Decline in Sulfatase Activity and ...
List of diseases (I)
... pulmonary haemosiderosis Idiopathic sclerosing mesenteritis Idiopathic thrombocytopenic purpura Iduronate 2-sulfatase ...
IDS
Indonesian educational institution Initiative for Democratic Socialism Institute for the German Language Iduronate-2-sulfatase ... a sulfatase enzyme associated with Hunter syndrome Index Catalogue of Visual Double Stars Integrated Deepwater System Program, ...
List of MeSH codes (D08)
... chondro-4-sulfatase MeSH D08.811.277.352.827.500 - iduronate sulfatase MeSH D08.811.277.352.897 - thiolester hydrolases MeSH ... n-acetylgalactosamine-4-sulfatase MeSH D08.811.277.352.827.070.250 - cerebroside-sulfatase MeSH D08.811.277.352.827.070.625 - ... steryl-sulfatase MeSH D08.811.277.352.827.180 - chondroitinases and chondroitin lyases MeSH D08.811.277.352.827.180.175 - ... sulfatases MeSH D08.811.277.352.827.070 - arylsulfatases MeSH D08.811.277.352.827.070.060 - ...
List of EC numbers (EC 3)
N-acetylgalactosamine-6-sulfatase EC 3.1.6.13: iduronate-2-sulfatase EC 3.1.6.14: N-acetylglucosamine-6-sulfatase EC 3.1.6.15: ... N-sulfoglucosamine-3-sulfatase EC 3.1.6.16: monomethyl-sulfatase EC 3.1.6.17: D-lactate-2-sulfatase EC 3.1.6.19: (R)-specific ... choline-sulfatase EC 3.1.6.7: cellulose-polysulfatase EC 3.1.6.8: cerebroside-sulfatase EC 3.1.6.9: chondro-4-sulfatase EC 3.1. ... steryl-sulfatase EC 3.1.6.3: glycosulfatase EC 3.1.6.4: N-acetylgalactosamine-6-sulfatase EC 3.1.6.5: deleted EC 3.1.6.6: ...