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InterPro: IPR001286 Glycoside hydrolase, family 59
Protein matches
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UniProtKB Matches: 47 proteins |
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Accession
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IPR001286 Glyco_hydro_59 |
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Type
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Family |
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Signatures
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InterPro Relationships
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Contains
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IPR013781 Glycoside hydrolase, subgroup, catalytic core
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GO Term annotation
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Process
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GO:0006683 galactosylceramide catabolic process
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Function
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GO:0004336 galactosylceramidase activity
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InterPro annotation
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 Entry Details in BioMart
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Abstract
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O-Glycosyl 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 glycosyl hydrolases, based on sequence similarity, has led to the definition of 85 different families [1, 2, 3]. This classification is available on the CAZy (CArbohydrate-Active EnZymes) web site [4]. Because the fold of proteins is better conserved than their sequences, some of the families can be grouped in clans.
Glycoside hydrolase family 59 GH59 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 [5, 6, 7]. GALC is responsible for the lysosomal catabolism of
certain galactolipids, including galactosylceramide and psychosine [5].
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Database links
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Pfam Clan: CL0058.12
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Publications
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1.
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Henrissat B, Callebaut I, Fabrega S, Lehn P, Mornon JP, Davies G.
Conserved catalytic machinery and the prediction of a common fold for several families of glycosyl hydrolases.
Proc. Natl. Acad. Sci. U.S.A. 92 7090-4 1995
[PubMed: 7624375]
http://www.pubmedcentral.nih.gov/picrender.fcgi?tool=EBI&pubmedid=7624375&action=stream&blobtype=pdf
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2.
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Davies G, Henrissat B.
Structures and mechanisms of glycosyl hydrolases.
Structure 3 853-9 1995
[PubMed: 8535779]
http://dx.doi.org/10.1016/S0969-2126(01)00220-9
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3.
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Bairoch A.
Classification of glycosyl hydrolase families and index of glycosyl hydrolase entries in SWISS-PROT.
1999
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4.
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Henrissat B, Coutinho PM.
Carbohydrate-Active Enzymes server.
1999
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5.
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Victoria T, Rafi MA, Wenger DA.
Cloning of the canine GALC cDNA and identification of the mutation causing globoid cell leukodystrophy in West Highland White and Cairn terriers.
Genomics 33 457-62 1996
[PubMed: 8661004]
http://dx.doi.org/10.1006/geno.1996.0220
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6.
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Luzi P, Rafi MA, Wenger DA.
Structure and organization of the human galactocerebrosidase (GALC) gene.
Genomics 26 407-9 1995
[PubMed: 7601472]
http://dx.doi.org/10.1016/0888-7543(95)80230-J
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7.
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Sakai N, Fukushima H, Inui K, Fu L, Nishigaki T, Yanagihara I, Tatsumi N, Ozono K, Okada S.
Human galactocerebrosidase gene: promoter analysis of the 5'-flanking region and structural organization.
Biochim. Biophys. Acta 1395 62-7 1998
[PubMed: 9434153]
http://dx.doi.org/10.1016/S0167-4781(97)00140-1
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InterPro 23.1
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