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We previously reported dysfunctional mitochondria in different <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> models. Now, using mitochondrial uncouplers and respiratory chain inhibitors, we have investigated with human fibroblasts a possible alteration in the selective degradation of damaged mitochondria (mitophagy) in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content>. By flow cytometry of MitoTracker\u2010labelled cells and measuring the levels of various mitochondrial proteins by western blot, we found in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> fibroblasts a partial impairment in the increased mitochondrial degradation produced by these treatments. In addition, colocalization of mitochondrial and lysosomal markers decreased in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> fibroblasts. All these results are consistent with a partial impairment in the induced autophagic degradation of dysfunctional mitochondria in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> fibroblasts. However, canonical recruitment of Parkin to mitochondria under these conditions remained unaffected in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> fibroblasts, and also in <jats:styled-content style=\"fixed-case\">SH<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">SY<\/jats:styled-content>5Y cells after malin and laforin overexpression. Neither mitochondrial localization nor protein levels of Bcl\u20102\u2010like protein 13, another component of the mitophagic machinery that operates under these conditions, were affected in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> fibroblasts. In contrast, although these treatments raised autophagy in both control and <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content> fibroblasts, this enhanced autophagy was clearly lower in the latter cells. Therefore, the autophagic degradation of altered mitochondria is impaired in <jats:styled-content style=\"fixed-case\">LD<\/jats:styled-content>, which is due to a partial defect in the autophagic response and not in the canonical mitophagy signalling pathways.<\/jats:p>","DOI":"10.1111\/febs.14468","type":"journal-article","created":{"date-parts":[[2018,4,12]],"date-time":"2018-04-12T10:13:13Z","timestamp":1523527993000},"page":"2071-2090","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Degradation of altered mitochondria by autophagy is impaired in Lafora disease"],"prefix":"10.1111","volume":"285","author":[{"given":"Marcos","family":"Lahuerta","sequence":"first","affiliation":[{"name":"Centro de Investigaci\u00f3n Pr\u00edncipe Felipe Valencia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen","family":"Aguado","sequence":"additional","affiliation":[{"name":"Centro de 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