Acid sphingomyelinase deficiency rescues mitochondrial dysfunction in gba-/- zebrafish (Danio rerio)
Objective: To determine the interaction between glucocerebrosidase deficiency and acid sphingomyelinase deficiency in a tractable vertebrate model system of Parkinson's disease. Background: Heterozygous glucocerebrosidase (GBA)…Ubiquitin carboxyl-terminal hydrolase 1 (UCHL1) -mediated ubiquitination attributed to localization of Mortalin to mitochondria
Objective: The pathogenic mechanism of UCHL1 in PD remains unclear. In this study, we aimed to investigate the role of UCHL1 in the pathogenesis of…Molecular Genetics and Functional Studies of LRRK2 Gene Variations in Parkinson’s Disease
Objective: To evaluate the function role of ROC domain in the brains of LRRK2 transgenic mice model and normal control mice. Background: Mutations in leucine-rich…Evaluation of mitochondrial dysfunction in fibroblasts and iPSC-derived dopaminergic neurons of patients affected by Multiple System Atrophy
Objective: The aim of the present study is to investigate mitochondrial functioning in fibroblasts and iPSC-derived dopaminergic neurons of patients affected by Multiple System Atrophy…Impaired stress-induced mitophagy in parkinsonian LRRK2(R1441G) knockin mutant mice
Objective: 1. Morphological changes and accumulation of ubiquitinated mitochondria in the striatum of aged leucine-rich-repeat kinase 2 (LRRK2)R1441G knockin mutant mice (Liu et al., 2014,…Citrus extract A protects dopaminergic neuron by modulating mitochondrial respiration and reactive oxygen species
Objective: This study aims to validate the protective effect of citrus extract A (CEA) against loss of dopamine neurons in PD by dual targeting of…CR6-interacting factor1 Deficiency in Dopamine Neurons Triggers Early-onset Parkinsonism in Mice
Objective: One candidate is CR6-interacting factor1 (CRIF1), which controls translation and membrane insertion of 13 mitochondrial proteins involved in oxidative phosphorylation. Here we determined the…Inhibition of mitochondrial complex I synthesis by chloramphenicol mitigates dopaminergic neuronal cell loss in PQ-induced parkinsonism
Objective: To find the potent drug for prevention of PD, we screened with above 1000 drugs in the PQ treated cells which currently used drug…Same cerebellar atrophy pattern in hyper- and hypokinetic movement disorders due to mitochondrial cytopathy
Objective: To describe the prevalence and phenotype of movement disorders (MDs) in patients with mitochondrial cytopathy (MC) and corresponding pattern of cerebral atrophy. Background: MCs…Parkinson’s disease-associated Miro1 mutants cause mitochondrial dysfunction
Objective: In the present study we dissected the role of the first PD-associated mutations in RhoT1, the gene encoding for Miro1. Background: Commonly involved pathogenic…
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