Dysregulated iron metabolism in C. elegans catp-6/ATP13A2 mutant impairs mitochondrial function.
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ABSTRACT:
SUBMITTER: Anand N
PROVIDER: S-EPMC7150649 | biostudies-literature | 2020 Jun
REPOSITORIES: biostudies-literature
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Dysregulated iron metabolism in C. elegans catp-6/ATP13A2 mutant impairs mitochondrial function.
Anand Nikhita N Holcom Angelina A Broussalian Michael M Schmidt Minna M Chinta Shankar J SJ Lithgow Gordon J GJ Andersen Julie K JK Chamoli Manish M
Neurobiology of disease 20200205
Mutations in the human ATP13A2 gene are associated with an early-onset form of Parkinson's disease (PD) known as Kufor Rakeb Syndrome (KRS). Patients with KRS show increased iron deposition in the basal ganglia, suggesting iron toxicity-induced neurodegeneration as a potential pathogenesis associated with the ATP13A2 mutation. Previously we demonstrated that functional losses of ATP13A2 disrupt the lysosomes ability to store excess iron, leading to reduce survival of dopaminergic neuronal cells. ...[more]
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