4ufh: Difference between revisions

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New page: ==Mouse Galactocerebrosidase complexed with iso-galacto-fagomine IGF== <StructureSection load='4ufh' size='340' side='right' caption='4ufh, resolution 2.16Å' scene...
 
m Protected "4ufh" [edit=sysop:move=sysop]
(No difference)

Revision as of 17:12, 26 March 2015

Mouse Galactocerebrosidase complexed with iso-galacto-fagomine IGFMouse Galactocerebrosidase complexed with iso-galacto-fagomine IGF

Structural highlights

4ufh is a 1 chain structure. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Ligands:, , , ,
Activity:Galactosylceramidase, with EC number 3.2.1.46
Resources:FirstGlance, OCA, RCSB, PDBsum

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]

References

  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

4ufh, resolution 2.16Å

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OCA