4ccd

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'''Unreleased structure'''
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{{STRUCTURE_4ccd| PDB=4ccd | SCENE= }}
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===STRUCTURE OF MOUSE GALACTOCEREBROSIDASE WITH D-GALACTAL: ENZYME- INTERMEDIATE COMPLEX===
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{{ABSTRACT_PUBMED_24297913}}
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The entry 4ccd is ON HOLD until Paper Publication
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==Disease==
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[[http://www.uniprot.org/uniprot/GALC_MOUSE GALC_MOUSE]] Defects in Galc are the cause of the 'twitcher' phenotype; an autosomal recessive leukodystrophy similar to the human disease (Krabbe disease). This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin.
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Authors: Hill, C.H., Graham, S.C., Read, R.J., Deane, J.E.
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==Function==
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[[http://www.uniprot.org/uniprot/GALC_MOUSE GALC_MOUSE]] Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.<ref>PMID:8769874</ref>
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Description: STRUCTURE OF MOUSE GALACTOCEREBROSIDASE WITH D-GALACTAL: ENZYME-INTERMEDIATE COMPLEX
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==About this Structure==
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[[4ccd]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CCD OCA].
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==Reference==
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<ref group="xtra">PMID:024297913</ref><references group="xtra"/><references/>
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[[Category: Galactosylceramidase]]
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[[Category: Deane, J E.]]
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[[Category: Graham, S C.]]
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[[Category: Hill, C H.]]
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[[Category: Read, R J.]]
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[[Category: D-galactal]]
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[[Category: Enzyme-intermediate complex]]
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[[Category: Galactosidase]]
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[[Category: Glycosyl hydrolase]]
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[[Category: Hydrolase]]
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[[Category: Krabbe disease]]
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[[Category: Lysosomal storage disease]]

Revision as of 13:40, 11 December 2013

Template:STRUCTURE 4ccd

Contents

STRUCTURE OF MOUSE GALACTOCEREBROSIDASE WITH D-GALACTAL: ENZYME- INTERMEDIATE COMPLEX

Template:ABSTRACT PUBMED 24297913

Disease

[GALC_MOUSE] Defects in Galc are the cause of the 'twitcher' phenotype; an autosomal recessive leukodystrophy similar to the human disease (Krabbe disease). This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin.

Function

[GALC_MOUSE] Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.[1]

About this Structure

4ccd is a 1 chain structure. Full crystallographic information is available from OCA.

Reference

  • Hill CH, Graham SC, Read RJ, Deane JE. Structural snapshots illustrate the catalytic cycle of beta-galactocerebrosidase, the defective enzyme in Krabbe disease. Proc Natl Acad Sci U S A. 2013 Dec 2. PMID:24297913 doi:http://dx.doi.org/10.1073/pnas.1311990110
  1. ↑ Sakai N, Inui K, Tatsumi N, Fukushima H, Nishigaki T, Taniike M, Nishimoto J, Tsukamoto H, Yanagihara I, Ozono K, Okada S. Molecular cloning and expression of cDNA for murine galactocerebrosidase and mutation analysis of the twitcher mouse, a model of Krabbe's disease. J Neurochem. 1996 Mar;66(3):1118-24. PMID:8769874

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