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Solution Structure of ZASP's PDZ domain

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

Overview

Z band alternately spliced PDZ-containing protein (ZASP) is a sarcomere Z disk protein expressed in human cardiac and skeletal muscle that is thought to be involved in a dominant familial dilated cardiomyopathy. The N-terminal PDZ domain of ZASP interacts with the C terminus of alpha-actinin-2, the major component of the Z disk, probably by forming a ternary complex with titin Z repeats. We have determined the structure of ZASP PDZ by NMR and showed that it is a classical class 1 PDZ domain that recognizes the carboxy-terminal sequence of an alpha-actinin-2 calmodulin-like domain with micromolar affinity. We also characterized the role of each component in the ternary complex ZASP/alpha-actinin-2/titin, showing that the alpha-actinin-2/ZASP PDZ interaction involves a binding surface distinct from that recognized by the titin Z repeats. ZASP PDZ structure was used to model other members of the enigma family by homology and to predict their abilities to bind alpha-actinin-2.

Disease

Known diseases associated with this structure: Cardiomyopathy, dilated OMIM:[605906], Cardiomyopathy, dilated, with left ventricular noncompaction OMIM:[605906], Myopathy, myofibrillar, ZASP-related OMIM:[605906]

About this Structure

1RGW is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Solution structure of ZASP PDZ domain; implications for sarcomere ultrastructure and enigma family redundancy., Au Y, Atkinson RA, Guerrini R, Kelly G, Joseph C, Martin SR, Muskett FW, Pallavicini A, Faulkner G, Pastore A, Structure. 2004 Apr;12(4):611-22. PMID:15062084

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