PMID: 9185184May 1, 1997Paper

Electron microscopic examination of basal lamina in Fukuyama congenital muscular dystrophy

Neuromuscular Disorders : NMD
H IshiiK Arahata

Abstract

By light microscopy we previously obtained immunocytochemical evidence for basal lamina (BL) abnormality of skeletal muscle in Fukuyama congenital muscular dystrophy (FCMD). To further elucidate the pathological involvement of the BL in FCMD, we examined by electron microscopy the skeletal muscle in 12 cases of FCMD, nine cases of age-matched neuromuscular diseases unrelated to FCMD, and a case of merosin-negative CMD (MCMD). We found that the BL of skeletal muscle fibres in all patients with FCMD and the MCMD patient had a thin, deranged and often disrupted appearance. These features were more prominent in large calibre (> 15 microns) fibres than in small calibre (approximately 8 microns) fibres. Replication of the BL was not observed in 11 of the 12 FCMD patients. Although the BL over the intact plasma membrane had occasional gaps, the plasma membrane under the disrupted BL was normal in some case. Our results indicate the presence of fragile BL which may precede plasma membrane damage in FCMD skeletal muscle.

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Citations

Sep 26, 2000·Muscle & Nerve·R D Cohn, K P Campbell
Apr 20, 2005·Neurobiology of Disease·Hiroki KurahashiTatsushi Toda
Nov 21, 2008·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Francesco MuntoniMartin Brockington
Mar 30, 2000·Pediatric Neurology·J VajsarL E Becker
Mar 29, 2000·Neuromuscular Disorders : NMD·T TodaY Nakamura
Jun 6, 2003·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Cecilia Jiménez-MallebreraFrancesco Muntoni
Feb 15, 2011·Human Molecular Genetics·Yung-Yao LinDerek L Stemple
Jun 18, 2002·Journal of Cellular and Molecular Medicine·Emilia Manole, Marilena Alexianu
Dec 14, 2006·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Jerry R MendellPaul T Martin
Nov 9, 2002·Lancet·Francesco MuntoniSusan C Brown
Oct 19, 2000·Neuropathology : Official Journal of the Japanese Society of Neuropathology·K Arahata
Mar 14, 2014·Expert Opinion on Biological Therapy·Aleksander TouznikToshifumi Yokota
Nov 2, 2013·Neurologic Clinics·Rohini CoorgMichael Wong
Aug 2, 2003·Congenital Anomalies·Tatsushi TodaKiichiro Matsumura
Sep 8, 2004·Pediatric Neurology·Tomoko YamamotoMakio Kobayashi
May 24, 2005·Neuromuscular Disorders : NMD·Tomohiro ChiyonobuTatsushi Toda
Sep 15, 2006·Nature Reviews. Molecular Cell Biology·Kay E Davies, Kristen J Nowak
Dec 2, 2005·Journal of Neuropathology and Experimental Neurology·Yuko SaitoShigeo Murayama
Feb 19, 2000·Microscopy Research and Technique·Y Miyagoe-SuzukiS Takeda
May 20, 2020·Disease Models & Mechanisms·Alec R NickollsCarsten G Bönnemann
Jan 18, 2006·Journal of Cell Science·Rita Barresi, Kevin P Campbell

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