Back to Search
Start Over
Freeze–fracture analysis of muscle plasma membrane in Becker's muscular dystrophy
- Source :
- Neuropathology and Applied Neurobiology. 20:487-494
- Publication Year :
- 1994
- Publisher :
- Wiley, 1994.
-
Abstract
- The intramembranous particle (IMP), orthogonal array (OA) and orthogonal array subunit particle (OASP) densities in skeletal muscle plasma membranes from eight patients with Becker's muscular dystrophy (BMD) were analysed by the freeze-fracture technique. The results showed almost normal IMP density with the significant decrease of OA and OASP densities in BMD. The group mean densities +/- SE of IMPs on the protoplasmic faces with and without OASPs, and on extracellular faces/microns 2 were 2137 +/- 207, 1839 +/- 68 and 895 +/- 108, respectively in controls; whereas those of BMD were 1989 +/- 259, 1837 +/- 203 and 900 +/- 239, respectively (P > 0.1 by two-tailed t-test). The group median density of OAs and their pits/microns 2 was 4.89 with mid-ranges (25-75% values of the counts) of 2.66-10.18 in controls; whereas that in BMD was 2.15 with mid-ranges of 1.14-4.31 (P < 0.01 by Wilcoxon rank-sum test). The group mean density +/- SE of OASPs in controls was 15.99 +/- 1.83; whereas that in BMD was 13.47 +/- 1.07 (P < 0.01 by two-tailed t-test). However, the diminution of OA and OASP densities in BMD muscle plasma membranes was not as severe as in Duchenne's muscular dystrophy. There was a relationship between OA density and clinical severity in BMD patients; the decrease of OA density in a severe BMD patient was more marked than that in mildly affected BMD patients. Therefore, it seems that marked depletion of OA density may lead to the severe disability in muscular dystrophies.
- Subjects :
- Adult
musculoskeletal diseases
medicine.medical_specialty
Histology
Adolescent
Becker's muscular dystrophy
Muscular Dystrophies
Pathology and Forensic Medicine
Physiology (medical)
Internal medicine
medicine
Extracellular
Freeze Fracturing
Humans
Clinical severity
Muscular dystrophy
Diminution
biology
Chemistry
Muscles
musculoskeletal, neural, and ocular physiology
Cell Membrane
Skeletal muscle
Anatomy
medicine.disease
Microscopy, Electron
Membrane
medicine.anatomical_structure
Endocrinology
Neurology
biology.protein
Neurology (clinical)
Dystrophin
Subjects
Details
- ISSN :
- 13652990 and 03051846
- Volume :
- 20
- Database :
- OpenAIRE
- Journal :
- Neuropathology and Applied Neurobiology
- Accession number :
- edsair.doi.dedup.....246b4a7baeb983287dc7ad6810daca18