2c0m
From Proteopedia
(New page: 200px<br /> <applet load="2c0m" size="450" color="white" frame="true" align="right" spinBox="true" caption="2c0m, resolution 2.50Å" /> '''APO FORM OF THE TPR...) |
|||
| Line 1: | Line 1: | ||
| - | [[Image:2c0m. | + | [[Image:2c0m.jpg|left|200px]]<br /><applet load="2c0m" size="350" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="2c0m" size=" | + | |
caption="2c0m, resolution 2.50Å" /> | caption="2c0m, resolution 2.50Å" /> | ||
'''APO FORM OF THE TPR DOMAIN OF THE PEX5P RECEPTOR'''<br /> | '''APO FORM OF THE TPR DOMAIN OF THE PEX5P RECEPTOR'''<br /> | ||
| Line 11: | Line 10: | ||
==About this Structure== | ==About this Structure== | ||
| - | 2C0M 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:// | + | 2C0M 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=2C0M OCA]. |
==Reference== | ==Reference== | ||
| Line 29: | Line 28: | ||
[[Category: zellweger syndrome]] | [[Category: zellweger syndrome]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 17:17:43 2008'' |
Revision as of 15:17, 15 February 2008
|
APO FORM OF THE TPR DOMAIN OF THE PEX5P RECEPTOR
Contents |
Overview
Peroxisomes require the translocation of folded and functional target, proteins of various sizes across the peroxisomal membrane. We have, investigated the structure and function of the principal import receptor, Pex5p, which recognizes targets bearing a C-terminal peroxisomal targeting, signal type 1. Crystal structures of the receptor in the presence and, absence of a peroxisomal target, sterol carrier protein 2, reveal major, structural changes from an open, snail-like conformation into a closed, circular conformation. These changes are caused by a long loop C terminal, to the 7-fold tetratricopeptide repeat segments. Mutations in residues of, this loop lead to defects in peroxisomal import in human fibroblasts. The, structure of the receptor/cargo complex demonstrates that the primary, receptor-binding site of the cargo is structurally and topologically, autonomous, enabling the cargo to retain its native structure and, function.
Disease
Known diseases associated with this structure: Adrenoleukodystrophy, neonatal OMIM:[600414], Zellweger syndrome OMIM:[600414]
About this Structure
2C0M is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Recognition of a functional peroxisome type 1 target by the dynamic import receptor pex5p., Stanley WA, Filipp FV, Kursula P, Schuller N, Erdmann R, Schliebs W, Sattler M, Wilmanns M, Mol Cell. 2006 Dec 8;24(5):653-63. PMID:17157249
Page seeded by OCA on Fri Feb 15 17:17:43 2008
