Research press release


Nature Medicine

Autophagy defect in muscle disease



ある型の遺伝性筋ジストロフィーのマウスを使って、P Bonaldoたちは、異常なミトコンドリアの残留と細胞死の原因が、筋細胞でのオートファジーの異常にあることを突き止めた。重要なのは、これらの細胞でオートファジーを活性化させると筋細胞が生き残るようになることで、筋ジストロフィーの新しい治療戦略に結びつく可能性がある。

Defects in autophagy — the degradation of damaged cellular organelles — are linked to muscular dystrophy, which is a disease in which muscle degenerates, according an article published this week in Nature Medicine.

Autophagy is crucial in the turnover of cell components and clearance of damaged organelles. Defects of this system have a role in various diseases, but little was known about autophagy in muscular dystrophy. Previous results had shown that some forms of muscular dystrophy are linked to abnormal mitochondria and spontaneous cell death, leading to muscle degeneration.

Using mice with a genetic form of muscular dystrophy, Paolo Bonaldo and his group show that the persistence of abnormal mitochondria and cell death are caused by defective autophagy in the muscle cell. Importantly, forced activation of autophagy in these cells restored muscle-cell survival in the mice, pointing to what could be a new therapeutic strategy against this disease.

doi: 10.1038/nm.2247


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