1ekg
From Proteopedia
(New page: 200px<br /> <applet load="1ekg" size="450" color="white" frame="true" align="right" spinBox="true" caption="1ekg, resolution 1.80Å" /> '''MATURE HUMAN FRATAX...) |
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caption="1ekg, resolution 1.80Å" /> | caption="1ekg, resolution 1.80Å" /> | ||
'''MATURE HUMAN FRATAXIN'''<br /> | '''MATURE HUMAN FRATAXIN'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1EKG is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http:// | + | 1EKG is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1EKG OCA]. |
==Reference== | ==Reference== | ||
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[[Category: mitochondrial]] | [[Category: mitochondrial]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 15:43:55 2008'' |
Revision as of 13:43, 15 February 2008
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MATURE HUMAN FRATAXIN
Contents |
Overview
Friedreich's ataxia, an autosomal recessive neurodegenerative disorder, characterized by progressive gait and limb ataxia, cardiomyopathy, and, diabetes mellitus, is caused by decreased frataxin production or function., The structure of human frataxin, which we have determined at 1.8-A, resolution, reveals a novel protein fold. A five-stranded, antiparallel, beta sheet provides a flat platform, which supports a pair of parallel, alpha helices, to form a compact alphabeta sandwich. A cluster of 12, acidic residues from the first helix and the first strand of the large, sheet form a contiguous anionic surface on the protein. The overall, protein structure and the anionic patch are conserved in eukaryotes, including animals, plants, and yeast, and in prokaryotes. Additional, conserved residues create an extended 1008-A(2) patch on a distinct, surface of the protein. Side chains of disease-associated mutations either, contribute to the anionic patch, help create the second conserved surface, or point toward frataxin's hydrophobic core. These structural findings, predict potential modes of protein-protein and protein-iron binding.
Disease
Known diseases associated with this structure: Friedreich ataxia OMIM:[606829], Friedreich ataxia with retained reflexes OMIM:[606829]
About this Structure
1EKG is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Crystal structure of human frataxin., Dhe-Paganon S, Shigeta R, Chi YI, Ristow M, Shoelson SE, J Biol Chem. 2000 Oct 6;275(40):30753-6. PMID:10900192
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