611. Alpha-Dystrobrevin-3 Prevents Myopathy and Restores Diaphragm Function in the Alpha-Dystrobrevin Null Mouse
نویسندگان
چکیده
منابع مشابه
Cellular and molecular properties of alpha-dystrobrevin in skeletal muscle.
The dystrophin glycoprotein complex (DGC) is a large multisubunit complex located throughout the sarcolemma of striated muscle fibers. This complex is critical for maintaining the structural integrity of muscle fibers during muscle contraction and also provides a scaffold for signaling molecules. Defects in some components of the DGC, such as dystrophin and sarcoglycans, disrupt the complex and...
متن کاملThe Role of Alpha-Dystrobrevin in Striated Muscle
Muscular dystrophies are a group of diseases that primarily affect striated muscle and are characterized by the progressive loss of muscle strength and integrity. Major forms of muscular dystrophies are caused by the abnormalities of the dystrophin glycoprotein complex (DGC) that plays crucial roles as a structural unit and scaffolds for signaling molecules at the sarcolemma. α-Dystrobrevin is ...
متن کاملIn Vivo Assessment of Calcium Influx in alpha-Dystrobrevin Knock-Out Mouse by Manganese-Enhanced MRI
Introduction Calcium (Ca) influx through the L-type Ca channel plays an important role in regulating cardiac function by directly regulating Ca release from sarcolemma reticulum. Recently, manganese (Mn), with its dual properties as a T1 contrast agent and a Ca surrogate that enters myocytes through Ca channels, has been used for in vivo delineation of changes in Ca influx under dramatically al...
متن کاملPlectin 1 links intermediate filaments to costameric sarcolemma through beta-synemin, alpha-dystrobrevin and actin.
In skeletal muscles, the sarcolemma is possibly stabilized and protected against contraction-imposed stress by intermediate filaments (IFs) tethered to costameric sarcolemma. Although there is emerging evidence that plectin links IFs to costameres through dystrophin-glycoprotein complexes (DGC), the molecular organization from plectin to costameres still remains unclear. Here, we show that plec...
متن کاملCharacterization of human alpha-dystrobrevin isoforms in HL-60 human promyelocytic leukemia cells undergoing granulocytic differentiation.
The biochemical properties and spatial localization of the protein alpha-dystrobrevin and other isoforms were investigated in cells of the human promyelocytic leukemia line HL-60 granulocytic differentiation as induced by retinoic acid (RA). Alpha-dystrobrevin was detected both in the cytosol and the nuclei of these cells, and a short isoform (gamma-dystrobrevin) was modified by tyrosine phosph...
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ژورنال
عنوان ژورنال: Molecular Therapy
سال: 2015
ISSN: 1525-0016
DOI: 10.1016/s1525-0016(16)34220-4