Preferential Subsarcolemmal Localization of Dystrophin and β-dystroglycan mRNA in Human Skeletal Muscles

The intracellular localization of dystrophin and β-dystroglycan mRNA in skeletal muscles of patients with Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) and normal subjects was examined by in situ hybridization using biotinylated oligonucleotide probes. These mRNAs were found p...

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 1997-01, Vol.56 (1), p.94-101
Main Authors: MITSUI, TAKAO, KAWAI, HISAOMI, SHANO, MASAYUKI, KAWAJIRI, MASAKAZU, KUNISHIGE, MAKOTO, SAITO, SHIRO
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Child
Child, Preschool
Cytoskeletal Proteins - genetics
Diseases of striated muscles. Neuromuscular diseases
Dystroglycans
Dystrophin - genetics
Humans
Image Processing, Computer-Assisted
In Situ Hybridization
Infant
Male
Medical sciences
Membrane Glycoproteins - genetics
Middle Aged
Muscle, Skeletal - chemistry
Muscle, Skeletal - ultrastructure
Muscular Dystrophies - classification
Muscular Dystrophies - metabolism
Muscular Dystrophies - pathology
Neurology
RNA, Messenger - analysis
Sarcolemma - chemistry
Sarcolemma - ultrastructure
Sequence Deletion
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Summary:The intracellular localization of dystrophin and β-dystroglycan mRNA in skeletal muscles of patients with Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) and normal subjects was examined by in situ hybridization using biotinylated oligonucleotide probes. These mRNAs were found preferentially in sarcolemma in the skeletal muscles of both normal subjects and affected patients. Quantitative analysis of mRNA signals demonstrated no prominent reduction of dystrophin or β-dystroglycan mRNA in DMD/BMD muscles. These results suggest that even mRNAs with deletions contain specific information that affects their localization, and the characteristic defect of dystrophin in DMD/BMD muscles seems to be caused mainly by the instability of dystrophin protein, as a post-transcriptional event.
ISSN:0022-3069
1554-6578
DOI:10.1097/00005072-199701000-00010