Connexin-based hemichannels are key factors in the pathological mechanism underlying dysferlinopathy


Dysferlinopathy onsets during the second and third decades of life, usually as progressive lower-limb weakness that later involves trunk and upper-limbs. Dysferlin is localized mainly in the sarcolemma and participates in membrane repair. However, in dysferlin-deficient (DD) mice the recovery of the membrane resealing function by expression of a mini-dysferlin does not arrest progressive muscular damage. The latter suggests the presence of dysferlin-dependent pathogenic mechanisms still unknown.


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