Housley, Michael P., Njaine, Brian, Ricciardi, Filomena, Stone, Oliver A., Hoelper, Soraya, Krueger, Marcus, Kostin, Sawa and Stainier, Didier Y. R. (2016). Cavin4b/Murcb Is Required for Skeletal Muscle Development and Function in Zebrafish. PLoS Genet., 12 (6). SAN FRANCISCO: PUBLIC LIBRARY SCIENCE. ISSN 1553-7404

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Abstract

Skeletal muscles provide metazoans with the ability to feed, reproduce and avoid predators. In humans, a heterogeneous group of genetic diseases, termed muscular dystrophies ( MD), lead to skeletal muscle dysfunction. Mutations in the gene encoding Caveolin-3, a principal component of the membrane micro-domains known as caveolae, cause defects in muscle maintenance and function; however it remains unclear how caveolae dysfunction underlies MD pathology. The Cavin family of caveolar proteins can form membrane remodeling oligomers and thus may also impact skeletal muscle function. Changes in the distribution and function of Cavin4/Murc, which is predominantly expressed in striated muscles, have been reported to alter caveolae structure through interaction with Caveolin-3. Here, we report the generation and phenotypic analysis of murcb mutant zebrafish, which display impaired swimming capacity, skeletal muscle fibrosis and T-tubule abnormalities during development. To understand the mechanistic importance of Murc loss of function, we assessed Caveolin-1 and 3 localization and found it to be abnormal. We further identified an in vivo function for Murc in Erk signaling. These data link Murc with developmental defects in T-tubule formation and progressive muscle dysfunction, thereby providing a new candidate for the etiology of muscular dystrophy.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Housley, Michael P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Njaine, BrianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ricciardi, FilomenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stone, Oliver A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoelper, SorayaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Krueger, MarcusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kostin, SawaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stainier, Didier Y. R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-273057
DOI: 10.1371/journal.pgen.1006099
Journal or Publication Title: PLoS Genet.
Volume: 12
Number: 6
Date: 2016
Publisher: PUBLIC LIBRARY SCIENCE
Place of Publication: SAN FRANCISCO
ISSN: 1553-7404
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
DUCHENNE MUSCULAR-DYSTROPHY; COILED-COIL PROTEIN; T-TUBULE BIOGENESIS; DILATED CARDIOMYOPATHY; GLYCOPROTEIN COMPLEX; EVANS BLUE; CAVEOLIN-3; MURC; SARCOLEMMA; ORGANIZATIONMultiple languages
Genetics & HeredityMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/27305

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