| Table of Contents |  |
|
CASE REPORT
|
| Degradation of myofibrillar proteins and inadequate antioxidants in selective muscle wasting of limb girdle muscular dystrophy |
| Rajakumar Dhanarajan1, Anilkumar B Patil2, Mathew Alexander3, Geeta Chacko4, Anna Oommen5 |
|
1Senior Research Fellow, Section of Neurochemistry, Department of Neurological Sciences, Christian Medical College, Vellore, Tamilnadu, India.
2Assistant Professor, Section of Neurology, Department of Neurological Sciences, Christian Medical College, Vellore, Tamilnadu, India. 3Professor, Section of Neurology, Department of Neurological Sciences, Christian Medical College, Vellore, Tamilnadu, India. 4Professor, Section of Neuropathology, Department of Neurological Sciences, Christian Medical College, Vellore, Tamilnadu, India. 5Senior Scientist Grade-I, Section of Neurochemistry, Department of Neurological Sciences, Christian Medical College, Vellore, Tamilnadu, India. |
|
doi:10.5348/ijcri-2011-06-37-CR-2
|
|
Address correspondence to: Dr. Rajakumar Dhanarajan Neurochemistry Laboratory Department of Neurological Sciences Christian Medical College Vellore 632 004 India Phone: 91-9790570526 Fax: 91-416-2232035 Email: dhanurajan@hotmail.com |
|
[HTML Abstract]
[PDF Full Text]
|
| How to cite this article: |
| Dhanarajan R, Patil AB, Alexander M, Chacko G, Oommen A. Degradation of myofibrillar proteins and inadequate antioxidants in selective muscle wasting of limb girdle muscular dystrophy. International Journal of Case Reports and Images 2011;2(6):6-11. |
|
Abstract
|
|
Introduction:
An unexplained feature of inherited muscular dystrophies is the wasting of selective muscle. Transcriptional signatures that differ between muscles may contribute to selective muscle wasting of muscular dystrophies. Biochemical signaling pathways involved in selective wasting have not been studied.
Case Report: This study examined signaling mechanisms that stimulate protein degradation, which may govern selective muscle wasting, in a patient with Miyoshi myopathy. Oxidative stress, nitric oxide signaling and protein degradation through the ubiquitin proteasome pathway were studied in relatively spared hamstring and wasted gastrocnemius muscle of the patient and in control muscle. Oxidative stress occurred in spared and wasted Miyoshi myopathic muscle. A strong anti-oxidant response was observed in spared muscle. Tyrosine nitration of 56kDa protein(s) was 2.3 and 5 fold higher in spared and wasted muscles respectively compared to normal. Nitrocysteinylated proteins were comparable between spared hamstring and normal muscle but reduced 35% in gastrocnemius muscle compared to normal. Ubiquitinylated proteins were increased 2.6 fold in the hamstring muscle and 1.6 fold in the gastrocnemius muscle compared to normal. The protein content of the hamstring and gastrocnemius muscles was reduced 43% and 37% respectively compared to normal. Myosin heavy chain and actin levels were normal in hamstring muscle but reduced nearly four fold in gastrocnemius muscle compared to normal. Conclusion: Oxidative and nitrosative stress and loss of actin and myosin were associated with selective muscle wasting of Miyoshi myopathy. A strong anti-oxidant response that protects myofibrillar proteins against degradation may spare muscle in Miyoshi myopathy. | |
|
Key Words:
Actin, Miyoshi myopathy, Myosin, Nitric oxide, Ubiquitin-proteasome pathway
| |
| [HTML Abstract] [PDF Full Text] |
|
Acknowledgement:
R. Dhanarajan is supported by a senior research fellowship of the Indian Council of Medical Research (Project No 5/4 -5/2/Neuro/2008). |
|
Author Contributions:
Rajakumar Dhanarajan - Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published Anilkumar B Patil - Conception and design, Drafting the article, Critical revision of the article, Final approval of the version to be published Mathew Alexader - Conception and design, Drafting the article, Critical revision of the article, Final approval of the version to be published Geeta Chacko - Conception and design, Drafting the article, Critical revision of the article, Final approval of the version to be published Anna Oommen - Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published |
|
Guarantor of submission:
The corresponding author is the guarantor of submission. |
|
Source of support:
None |
|
Conflict of interest:
The author(s) declare no conflict of interests |
|
Copyright:
© Rajakumar Dhanarajan et al. 2011; This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any means provided the original authors and original publisher are properly credited. (Please see Copyright Policy for more information.) |
|
|