New Insights into the Roles of Xin Repeat-Containing Proteins in Cardiac Development, Function, and Disease

Qinchuan Wang; Jenny Li-Chun Lin; Albert J Erives; Cheng-I Lin; Jim Jung-Ching Lin

doi:10.1016/B978-0-12-800180-6.00003-7

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New Insights into the Roles of Xin Repeat-Containing Proteins in Cardiac Development, Function, and Disease

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Peer reviewed

New Insights into the Roles of Xin Repeat-Containing Proteins in Cardiac Development, Function, and Disease

Qinchuan Wang, Jenny Li-Chun Lin, Albert J Erives, Cheng-I Lin and Jim Jung-Ching Lin

International Review of Cell and Molecular Biology, pp.89-128

Elsevier Science & Technology

2014

DOI: 10.1016/B978-0-12-800180-6.00003-7

PMCID: PMC4857591

PMID: 24725425

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Abstract

Since the discovery of Xin repeat-containing proteins in 1996, the importance of Xin proteins in muscle development, function, regeneration, and disease has been continuously implicated. Most Xin proteins are localized to myotendinous junctions of the skeletal muscle and also to intercalated discs (ICDs) of the heart. The Xin gene is only found in vertebrates, which are characterized by a true chambered heart. This suggests that the evolutionary origin of the Xin gene may have played a key role in vertebrate origins. Diverse vertebrates including mammals possess two paralogous genes, Xinα (or Xirp1) and Xinβ (or Xirp2), and this review focuses on the role of their encoded proteins in cardiac muscles. Complete loss of mouse Xinβ (mXinβ) results in the failure of forming ICD, severe growth retardation, and early postnatal lethality. Deletion of mouse Xinα (mXinα) leads to late-onset cardiomyopathy with conduction defects. Molecular studies have identified three classes of mXinα-interacting proteins: catenins, actin regulators/modulators, and ion-channel subunits. Thus, mXinα acts as a scaffolding protein modulating the N-cadherin-mediated adhesion and ion-channel surface expression. Xin expression is significantly upregulated in early stages of stressed hearts, whereas Xin expression is downregulated in failing hearts from various human cardiomyopathies. Thus, mutations in these Xin loci may lead to diverse cardiomyopathies and heart failure.

Cortactin

KChIP2

Intercalated disc formation

Xirp

β-Catenin

Delayed K+ rectifier current (Ik,slow1)

Cardiomyopathy with conduction defect

Transient K+ outward current (Ito,f)

Details

Title: Subtitle: New Insights into the Roles of Xin Repeat-Containing Proteins in Cardiac Development, Function, and Disease
Creators: Qinchuan Wang - Department of Biology, University of Iowa, Iowa City, Iowa, USA
Jenny Li-Chun Lin - Department of Biology, University of Iowa, Iowa City, Iowa, USA
Albert J Erives - Department of Biology, University of Iowa, Iowa City, Iowa, USA
Cheng-I Lin - Institute of Physiology, National Defense Medical Center, Taipei, Taiwan, ROC
Jim Jung-Ching Lin - Department of Biology, University of Iowa, Iowa City, Iowa, USA
Resource Type: Book chapter
Publication Details: International Review of Cell and Molecular Biology, pp.89-128
DOI: 10.1016/B978-0-12-800180-6.00003-7
PMID: 24725425
PMCID: PMC4857591
NLM abbreviation: Int Rev Cell Mol Biol
ISSN: 1937-6448
Publisher: Elsevier Science & Technology
Language: English
Date published: 2014
Academic Unit: Biology
Record Identifier: 9983991950802771

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