Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells.

Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Research Abstract Details 

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Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Abstract Text:

Romesh A Draviam,Bing Wang,Juan Li,Xiao Xiao,Simon C Watkins,

Dystrophin is a critical muscle cell structural protein which when deficient results in Duchenne muscular dystrophy. Recently miniature versions of the dystrophin gene have been constructed that ameliorate the pathology in mouse models. To characterize mini-dystrophin's incorporation into the dystrophin protein complex in living cells, two fusion proteins were constructed where mini-dystrophin is fused to the N- or C-terminus of an enhanced green fluorescent protein reporter gene. Both fusion proteins correctly localize at the plasma membrane in vitro and in vivo. Live cell microscopy establishes that mini-dystrophin translocates directly to the PM of differentiating muscle cells, within 4 h of expression. Latrunculin A treatment, actin and beta-dystroglycan binding domain deletion constructs, and co-immunoprecipitation assays demonstrate that mini-dystrophin is firmly anchored to the sarcolemma primarily through its connections to beta-dystroglycan, mimicking effects seen with wild type dystrophin. Furthermore, point mutations made within the putative beta-dystroglycan anchoring ZZ domain of mini-dystrophin result in an ablation of beta-dystroglycan binding and a nuclear translocation of the protein. These results demonstrate that mini-dystrophin is efficiently bound and incorporated into the dystrophin protein complex, via beta-dystroglycan in living cells, similarly to the full length dystrophin protein.

Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Publishing Authors By Initials

RA Draviam,B Wang,J Li,X Xiao,SC Watkins,

For similar thiazoles: thiazolidines research abstracts see: thiazoles: thiazolidines research

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Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Journal Published:

PUBLICATION TYPE: Research Support, N.I.H., Extr

Journal: Journal of muscle research and cell motility

VOLUME: 27

Page Numbers: 53-67

Journal Abbreviation: J. Muscle Res. Cell. Motil.

ISSN: 0142-4319

DAY: 23

MONTH: 02

YEAR: 2006

Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Information

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LANGUAGE: eng

NlmUniqueID: 8006298

Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Keywords Mesh Terms:

KEYWORDS: Thiazolidines

MESH TERMS: pharmacology

Chemical & Substance for Abstract: Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells. Information

Substance Name: latrunculin A

Registry Number: 76343-93-6

Grant and Affiliation Information for Mini-dystrophin efficiently incorporates into the dystrophin protein complex in living cells.

AFFILIATION: Department of Cell Biology and Molecular Physiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA. romesh@ pitt.edu

Country: Netherlands

AGENCY: United States NIAMS

GRANT: U54-AR050733

ACRONYM: AR

MEDLINETA: J Muscle Res Cell Motil

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