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Article

Autophagy induction rescues muscular dystrophy.

Autophagy - 1 Apr 2011

Grumati Paolo, Coletto Luisa, Sandri Marco, Bonaldo Paolo

Abstract excerpt

Collagen VI is an extracellular matrix protein forming a microfibrillar network in the endomysium of skeletal muscles. In humans, mutations in any of the three genes coding for collagen VI cause several skeletal muscle diseases, including Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD). Collagen VI null (Col6a1(-/-)) mice display a myopathic phenotype resembling that of BM and UCMD...

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Identifiers

PMID: 21543891
DOI: 10.4161/auto.7.4.14392

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