2c0l

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spinqualitylabelsall models PDB ID 2c0l Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image
, resolution 2.30Å
Coordinates:	save as pdb, mmCIF, xml

TPR DOMAIN OF HUMAN PEX5P IN COMPLEX WITH HUMAN MSCP2

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

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

2C0L is a Protein complex structure of sequences 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

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