CDD: smart00033.5, CH
PFAM: PF00307
InterPro: IPR001715
SMART: SM0033
 

Calponin Homology (CH) Domain From Human Beta Spectrin
Authors:   K. Djinovic Carugo, S. Banuelos, M. Saraste
pdb file: 1dro
jpeg generated from pdb file 1dro using Chime
 

Description

The CH domain is an approximately 100 residue domain containing 4 a-helices bundled in an aa-motif type arrangement with three of the helices roughly parallel and the fourth perpendicular to those helices. These are often seen in dimeric pairs, but can also occur as a lone domain as well. Loops connecting the alpha helices may vary in size.

Calponin Homology Domains act as structural and signal pathway proteins serving as actin-binding domains within these proteins. These domains cross-link actin filaments into bundles networks that allow them to function in tissues such as muscle. There is evidence that CH domains may also play transcriptional regulatory roles in signal transduction proteins such as Vav, a proto-oncogene (PubMed: 12400014).
 

Examples of proteins containing the CH domain

Vav
TCR-mediated signaling with MHC complex in selection of thymocytes.
PubMed: 10544657
LocusLink: 25156

Utrophin
Lateral binding and stabilization of actin filaments.  Homologous to dystrophin.
PubMed: 12006649
LocusLink: 22288

Alpha Actinin
Involved in Signaling processes that promote development of actin microspikes and cell separation.
PubMed: 12356918
LocusLink 31166

Spectrin
A fibrous protein of the cytoskeleton existing in heterodimer form that interacts with actin proteins.
PubMed: 12064945
LocusLink: 6711

Calponin
A thin filament-associated protein involved in regulation and modulation of smooth muscle contraction.
PubMed: 11805159
LocusLink: 1264

Human Disease

X-Linked Dilated Cardio Myopathy (OMIM: 302045)

X-Linked dilated cardiomyopathy (XLDC) is a classification of dystrophinopathy characterized by preferential myocardial involvement without any signs of skeletal myopathy. This is a familial myocardial disease that results in lethal congestive heart failure in males while in their teens or early twenties. Many sufferers of this disease carry mutations in the dystrophin gene (LocusLink: 1756) , specifically caused by insertions or deletions in the N-terminal region coding for the calponin homology domain.(X-linked dilated cardiomyopathy (XLDC) and the dystrophin gene).
 
 

Submitted by:
Josh Johnson and Melissa Ober
Cell Biology Class
Shippensburg University
12/5/02

Edited by:

William J. Patrie  12/15/02