{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T08:16:57Z","timestamp":1789805817632,"version":"4.0.1"},"reference-count":47,"publisher":"Wiley","license":[{"start":{"date-parts":[[2016,1,1]],"date-time":"2016-01-01T00:00:00Z","timestamp":1451606400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"The South African Medical Council"},{"DOI":"10.13039\/501100001321","name":"National Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001321","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004513","name":"Harry Crossley Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004513","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Parkinson's Disease"],"published-print":{"date-parts":[[2016]]},"abstract":"<jats:p>Mutations in the<jats:italic>parkin<\/jats:italic>gene are the most common cause of early-onset Parkinson\u2019s disease (PD). Parkin, an E3 ubiquitin ligase, is involved in respiratory chain function, mitophagy, and mitochondrial dynamics. Human cellular models with<jats:italic>parkin<\/jats:italic>null mutations are particularly valuable for investigating the mitochondrial functions of parkin. However, published results reporting on patient-derived<jats:italic>parkin<\/jats:italic>-mutant fibroblasts have been inconsistent. This study aimed to functionally compare<jats:italic>parkin<\/jats:italic>-mutant fibroblasts from PD patients with wild-type control fibroblasts using a variety of assays to gain a better understanding of the role of mitochondrial dysfunction in PD. To this end, dermal fibroblasts were obtained from three PD patients with homozygous whole exon deletions in<jats:italic>parkin<\/jats:italic>and three unaffected controls. Assays of mitochondrial respiration, mitochondrial network integrity, mitochondrial membrane potential, and cell growth were performed as informative markers of mitochondrial function. Surprisingly, it was found that mitochondrial respiratory rates were markedly higher in the<jats:italic>parkin<\/jats:italic>-mutant fibroblasts compared to control fibroblasts (<jats:italic>p<\/jats:italic>= 0.0093), while exhibiting more fragmented mitochondrial networks (<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\"><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn fontstyle=\"italic\">0.0304<\/mml:mn><\/mml:math>). Moreover, cell growth of the<jats:italic>parkin<\/jats:italic>-mutant fibroblasts was significantly higher than that of controls (<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M2\"><mml:mi>p<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn fontstyle=\"italic\">0.0001<\/mml:mn><\/mml:math>). These unanticipated findings are suggestive of a compensatory mechanism to preserve mitochondrial function and quality control in the absence of parkin in fibroblasts, which warrants further investigation.<\/jats:p>","DOI":"10.1155\/2016\/1819209","type":"journal-article","created":{"date-parts":[[2016,3,9]],"date-time":"2016-03-09T02:18:46Z","timestamp":1457489926000},"page":"1-11","source":"Crossref","is-referenced-by-count":18,"title":["Altered Mitochondrial Respiration and Other Features of Mitochondrial Function in<i>Parkin<\/i>-Mutant Fibroblasts from Parkinson\u2019s Disease Patients"],"prefix":"10.1155","volume":"2016","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3946-6663","authenticated-orcid":true,"given":"William","family":"Haylett","sequence":"first","affiliation":[{"name":"Division of Molecular Biology and Human Genetics, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South 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