Galactosylceramidase
From Proteopedia
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<StructureSection load='3zr5' size='340' side='right' caption='Galactosylceramidase scene=''> | <StructureSection load='3zr5' size='340' side='right' caption='Galactosylceramidase scene=''> | ||
| - | Galactosylceramidase (GALC) is a hydrolase <ref>RCSB Protein Data Bank - RCSB PDB - 3ZR5 Structure Summary. (n.d.). RCSB Protein Data Bank - RCSB PDB - 3ZR5 Structure Summary. Retrieved June 3, 2014, from www.rcsb.org DOI:10.2210/pdb3zr5/pdb</ref> that removes galactose from ceramide derivatives ('''reference'''). Galactosylceramidase in humans is encoded by the gene GALC, and mutations in this gene are associated with Krabbe disease, or globoid cell leukodystrophy <ref name=Deane>PMID: 21876145</ref>. | + | Galactosylceramidase (GALC) (also known as galactocerebrosidase) is a hydrolase <ref>RCSB Protein Data Bank - RCSB PDB - 3ZR5 Structure Summary. (n.d.). RCSB Protein Data Bank - RCSB PDB - 3ZR5 Structure Summary. Retrieved June 3, 2014, from www.rcsb.org DOI:10.2210/pdb3zr5/pdb</ref> that removes galactose from ceramide derivatives ('''reference'''). Galactosylceramidase in humans is encoded by the gene GALC, and mutations in this gene are associated with Krabbe disease, or globoid cell leukodystrophy <ref name=Deane>PMID: 21876145</ref>. |
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==Structure== | ==Structure== | ||
X-ray diffraction data (<scene name='58/587874/Galactosylceramidase/1'>default scene</scene>) from mouse models indicates that GALC is an estimated 77 kDa protein consisting of 656 residues, which form a secondary structure containing 12 α-helices and 41 β-strands. Each β-strand contains three to eleven residues. | X-ray diffraction data (<scene name='58/587874/Galactosylceramidase/1'>default scene</scene>) from mouse models indicates that GALC is an estimated 77 kDa protein consisting of 656 residues, which form a secondary structure containing 12 α-helices and 41 β-strands. Each β-strand contains three to eleven residues. | ||
| - | Use this link to<scene name="/12/3456/Sample/1">color</scene> by group, and this link to view a <scene name="/12/3456/Sample/2">a transparent representation</scene> of the protein. | + | Use this link to <scene name="/12/3456/Sample/1">color</scene> by group, and this link to view a <scene name="/12/3456/Sample/2">a transparent representation</scene> of the protein. |
== Function == | == Function == | ||
Revision as of 18:37, 3 June 2014
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References
- ↑ RCSB Protein Data Bank - RCSB PDB - 3ZR5 Structure Summary. (n.d.). RCSB Protein Data Bank - RCSB PDB - 3ZR5 Structure Summary. Retrieved June 3, 2014, from www.rcsb.org DOI:10.2210/pdb3zr5/pdb
- ↑ 2.0 2.1 Deane JE, Graham SC, Kim NN, Stein PE, McNair R, Cachon-Gonzalez MB, Cox TM, Read RJ. Insights into Krabbe disease from structures of galactocerebrosidase. Proc Natl Acad Sci U S A. 2011 Sep 13;108(37):15169-73. Epub 2011 Aug 29. PMID:21876145 doi:10.1073/pnas.1105639108
- ↑ Belleri M, Ronca R, Coltrini D, Nico B, Ribatti D, Poliani PL, Giacomini A, Alessi P, Marchesini S, Santos MB, Bongarzone ER, Presta M. Inhibition of angiogenesis by beta-galactosylceramidase deficiency in globoid cell leukodystrophy. Brain. 2013 Sep;136(Pt 9):2859-75. doi: 10.1093/brain/awt215. PMID:23983033 doi:http://dx.doi.org/10.1093/brain/awt215
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