Eur Rev Med Pharmacol Sci 2016; 20 (17): 3683-3687

Dystroglycan induced muscular dystrophies – a review

Q.-Z. Zhang

Department of Neurology, Provincial Hospital Affiliated to Shandong University, Jinan, Shandong, China. 386860155@qq.com


Dystroglycanopathies are muscular dystrophies caused by mutations in genes involved the in O-linked glycosylation of α-dystroglycan. Severe forms of these conditions result in abnormalities in exhibit brain and ocular developmental too, in addition to muscular dystrophy. The full spectrum of developmental pathology is caused mainly by loss of dystroglycan from Bergmann glia. Moreover, cognitive deficits are constant features of severe forms of dystroglycanopathies. However, the precise molecular mechanism leading to neuronal dysfunction in these diseases is not fully known yet. The present review article will discuss the importance of dystroglycan in cerebellar development and associated pathological states.

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To cite this article

Q.-Z. Zhang
Dystroglycan induced muscular dystrophies – a review

Eur Rev Med Pharmacol Sci
Year: 2016
Vol. 20 - N. 17
Pages: 3683-3687