A nuclear-encoded mitochondrial complex I subunit gene, NDUFV2, is one of the candidate genes in this locus, since the possible pathophysiological significance of mitochondrial dysfunction in bipolar disorder has been suggested. The objective of our study was to clarify the association between the ...
Parkinson’s disease (PD) is a heterogeneous, clinicopathologically defined neurodegenerative disorder, with an age-dependent incidence and a rapidly rising global prevalence1. More than 95% of affected individuals, in most populations, have idiopathic forms of PD (iPD), i.e., not of monogenic o...
Complex I dysfunction is the most common oxidative phosphorylation (OXPHOS) disorder in humans and defects in the complex I assembly process are often observed. This assembly process has been difficult to characterize because of its large size, the lack of a high resolution structure for complex I...
FAT IMPROVES MITOCHONDRIAL PERFORMANCE IN A RESPIRATORY CHAIN DISORDER(COMPLEX I DEFICIENCY). |[dagger]| 848Pediatric Research publishes original papers, invited reviews, and commentaries on the etiologies of diseases of children and disorders of development, extending from molecular biology to epidemiology...
The NDUFV1 knockdown-induced blockage of insulin signaling was released by protein-tyrosine phosphatase 1B knockdown in C2C12 myotubes, and we found that NDUFV1 or SIRT1 knockdown did not affect mitochondria biogenesis during C2C12 myogenesis. Based on these data, we can conclude that complex I ...
1.mitochondrion线粒体 2.Lou Gehrig’s disease卢·格里克氏症;肌萎缩性脊髓侧索硬化症 3.autosomalrecessive inheritance常染色体隐性遗传 4. dominant显性的 5.autism spectrum disorder自闭症谱系障碍 6.cramp抽筋 7.lactic acidosis乳酸性酸中毒 8.dementia痴呆 ...
Mitochondrial metabolic disorder is one of the important pathogeneses of DCM [44], while Pdk4, Hmgcs2, Decr1, and Ivd are enzymes essential for mitochondrial metabolism. In DCM, the most significant metabolic disorders in myocardial tissues are decreased glucose utilization and increased fatty acid...
which helped the cells functional oxygen consumption and respiration. In addition, mitochondrial transfer helped ρ0 cells restore their cellular behaviour, including nonadherent proliferation, aerobic viability, and OXPHOS dependent cellular motility [83]. Moreover, ETC complex V-inhibitor-sensitive ATP ...
The transfer of whole mitochondria that occurs during cell contact has been found to support cancer progression. However, the regulatory role of mitochondria alone is difficult to elucidate due to the complex microenvironment. Currently, mitochondrial tr
Recently, mutations in nuclear complex I genes have been identified in patients with Leigh syndrome. The disease-causing genes (NDUFS8 [MIM 602141], NDUFS4 [MIM 602694], NDUFS7 [MIM 601825], and NDUFV1 [MIM 161015]) (Loeffen et al. 2000) are highly conserved across species and play ...