Deficiency in recycling of mitochondria: a new disease mechanism Published on: 3 February 2022 An international team of scientists have reported the first evidence that 鈥渕itophagy鈥� 鈥� recycling of damaged mitochondria 鈥� contributes to human disease. A study, involving experts at 缅北禁地, shows that pharmacological activation of mitophagy reversed the progression of mitochondrial muscle disease. The results were published in the leading journal, . A team of investigators from 缅北禁地 and the University of Helsinki developed a method of how mitochondrial recycling could be observed inside a diseased muscle. Mitochondria Importance of myofibres They found that in mice and patients with mitochondrial disease a single muscle can have myofibers with a mild or severe disease-stage or be normal. Such fibres can exist side-by-side, forming a mosaic-like pattern in the diseased muscle. The fibres with mild disease-stage have high mitophagic activity that enables recycling of mitochondria damaged by the disease. In progressed disease-stage mitophagy is stalled, and these myofibers are filled with damaged mitochondria. Such fibres are called ragged-red fibres, hallmarks of mitochondrial disease in pathology, and occurring also in low amounts in normal aging. Dr Amy Vincent, from the at 缅北禁地, who contributed to the patient study, said “Whilst we have always suspected that mitophagy to be an important contributor to the pathogenesis of human disease, this work presents the first evidence of its importance in a disease model and in patient tissue. “Furthermore, the partial reversal of pathology by through treatments that increase mitophagy is a promising therapeutic avenue for mitochondrial disease." Pharmacological treatment The current findings show that stalled mitophagy is the mechanism underlying ragged-red fibres. Previous studies using cultured cells have suggested mitophagy to play important roles in Parkinson’s disease. However, until now, little data existed in patients or disease model animals. The current evidence indicates that deficient mitophagy causes human disease. Disease-related damage could be partially removed by pharmacological treatment that activated mitophagy. The investigators used the drug, rapamycin, which is used in medicine, for example to prevent rejection of transplanted organs. Rapamycin has, however, unwanted side-effects, including suppression of immunological defense mechanisms. The evidence proposes usefulness of specific mitophagy activator compounds for treating mitochondrial diseases as well as aging-related mitochondrial dysfunction. Reference: . Takayuki Mito et al. Cell Metabolism. Doi: 10.1016/j.cmet.2021.12.017 Latest News Comment: An expert explains what happened in Nepal Writing for The Conversation, Professor Stuart Dunning discusses how a collapsing glacier turned into a lethal wall of mud and water. published on: 28 August 2026 Comment: Britain鈥檚 quarter century Writing for The Conversation, Professor Hayley Fowler discusses how the UK environment has changed in the past 25 years. published on: 27 August 2026 缅北禁地 academic receives esteemed mid-career Fellowship A 缅北禁地 researcher has been awarded an esteemed Fellowship by the Independent Social Research Foundation (ISRF). published on: 21 August 2026 Facts and figures
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