Glycoside hydrolase family 59
| Glycosyl hydrolase family 59 | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| Symbol | Glyco_hydro_59 | ||||||||
| Pfam | PF02057 | ||||||||
| Pfam clan | CL0058 | ||||||||
| InterPro | IPR001286 | ||||||||
| OPM superfamily | 125 | ||||||||
| OPM protein | 3zr5 | ||||||||
| CAZy | GH59 | ||||||||
| |||||||||
In molecular biology, glycoside hydrolase family 59 is a family of glycoside hydrolases.
Glycoside hydrolases EC 3.2.1. are a widespread group of enzymes that hydrolyse the glycosidic bond between two or more carbohydrates, or between a carbohydrate and a non-carbohydrate moiety. A classification system for glycoside hydrolases, based on sequence similarity, has led to the definition of >100 different families.[1][2][3] This classification is available on the CAZy(http://www.cazy.org/GH1.html) web site,[4] and also discussed at CAZypedia, an online encyclopedia of carbohydrate active enzymes.[5]
Glycoside hydrolase family 59 CAZY GH_59 comprises enzymes with only one known activity; galactocerebrosidase (EC 3.2.1.46). Globoid cell leukodystrophy (Krabbe disease) is a severe, autosomal recessive disorder that results from deficiency of galactocerebrosidase (GALC) activity.[6][7][8] GALC is responsible for the lysosomal catabolism of certain galactolipids, including galactosylceramide and psychosine.[6]
References
- ↑ Henrissat B, Callebaut I, Mornon JP, Fabrega S, Lehn P, Davies G (1995). "Conserved catalytic machinery and the prediction of a common fold for several families of glycosyl hydrolases". Proc. Natl. Acad. Sci. U.S.A. 92 (15): 7090–7094. doi:10.1073/pnas.92.15.7090. PMC 41477
. PMID 7624375. - ↑ Henrissat B, Davies G (1995). "Structures and mechanisms of glycosyl hydrolases". Structure. 3 (9): 853–859. doi:10.1016/S0969-2126(01)00220-9. PMID 8535779.
- ↑ Bairoch, A. "Classification of glycosyl hydrolase families and index of glycosyl hydrolase entries in SWISS-PROT". 1999.
- ↑ Henrissat, B. and Coutinho P.M. "Carbohydrate-Active Enzymes server". 1999.
- ↑ CAZypedia, an online encyclopedia of carbohydrate-active enzymes.
- 1 2 Rafi MA, Wenger DA, Victoria T (1996). "Cloning of the canine GALC cDNA and identification of the mutation causing globoid cell leukodystrophy in West Highland White and Cairn terriers". Genomics. 33 (3): 457–462. doi:10.1006/geno.1996.0220. PMID 8661004.
- ↑ Luzi P, Rafi MA, Wenger DA (1995). "Structure and organization of the human galactocerebrosidase (GALC) gene". Genomics. 26 (2): 407–409. doi:10.1016/0888-7543(95)80230-J. PMID 7601472.
- ↑ Fukushima H, Inui K, Fu L, Nishigaki T, Yanagihara I, Tatsumi N, Ozono K, Okada S, Sakai N (1998). "Human galactocerebrosidase gene: promoter analysis of the 5'-flanking region and structural organization". Biochimica et Biophysica Acta (BBA) - Gene Structure and Expression. 1395 (1): 62–67. doi:10.1016/S0167-4781(97)00140-1. PMID 9434153.
This article incorporates text from the public domain Pfam and InterPro IPR001286