NDLI: Cyclosporine A in Ullrich Congenital Muscular Dystrophy: Long-Term Results

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Cyclosporine A in Ullrich Congenital Muscular Dystrophy: Long-Term Results

Content Provider	PubMed Central
Author	Merlini, Luciano Sabatelli, Patrizia Armaroli, Annarita Gnudi, Saverio Angelin, Alessia Paolo, Grumati Michelini, Maria Elena Andrea, Franchella Gualandi, Francesca Bertini, Enrico Maraldi, Nadir Mario Ferlini, Alessandra Paolo, Bonaldo Bernardi, Paolo
Copyright Year	2011
Abstract	Six individuals with Ullrich congenital muscular dystrophy (UCMD) and mutations in the genes-encoding collagen VI, aging 5–9, received 3–5 mg/kg of cyclosporine A (CsA) daily for 1 to 3.2 years. The primary outcome measure was the muscle strength evaluated with a myometer and expressed as megalimbs. The megalimbs score showed significant improvement (P = 0.01) in 5 of the 6 patients. Motor function did not change. Respiratory function deteriorated in all. CsA treatment corrected mitochondrial dysfunction, increased muscle regeneration, and decreased the number of apoptotic nuclei. Results from this study demonstrate that long-term treatment with CsA ameliorates performance in the limbs, but not in the respiratory muscles of UCMD patients, and that it is well tolerated. These results suggest considering a trial of CsA or nonimmunosuppressive cyclosporins, that retains the PTP-desensitizing properties of CsA, as early as possible in UCMD patients when diaphragm is less compromised.
Related Links	http://dx.doi.org/10.1155/2011/139194
Starting Page	139194
File Format	PDF
ISSN	19420900
e-ISSN	19420994
Journal	Oxidative Medicine and Cellular Longevity
Volume Number	2011
Language	English
Publisher	Hindawi Publishing Corporation
Publisher Date	2011-01-01
Access Restriction	Open
Rights Holder	Hindawi Publishing Corporation
Subject Keyword	Cell Biology Biochemistry Ageing Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Cell Biology Aging Medicine Biochemistry

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