To select a robust marker for neuronal CI immunodetection, we tested antibodies against different CI subunits across the functional domains of the complex26in the SNpc of 5 individuals with iPD and 3 controls. All tested subunits exhibited a similar pattern and severity of neuronal CI deficiency,...
Mitochondrial Complex I (MCI) deficiency has been abundantly observed in Parkinson’s disease (PD); however, it remained elusive if this was a consequence of the disease defects or rather disease-causing. Recent work in Nature by Gonzalez-Rodriguez and colleagues discovered MCI dysfunction to be s...
Disruption of mitochondrial complex I induces progressive parkinsonism Article 03 November 2021 Crif1 deficiency in dopamine neurons triggers early-onset parkinsonism Article 30 August 2023 References Galluzzi, L., Kepp, O. & Kroemer, G. Mitochondria: master regulators of danger signalling. Nat....
Rather, TMBIM5 deficiency attenuates K+/H+ exchange activity [39]. Alternatively, TMBIM5 was proposed as a Ca2+/H+ antiporter [40,41]. In particular, T. Langer's lab proposed TMBIM5 as an interactor of the m-AAA protease AFG3L2 and as a Ca2+/H+ exchanger allowing Ca2+ efflux ...
Mitochondrial complex I deficiency in Parkinson’s disease. J Neurochem. 1990;543:823–7. Article Google Scholar Drouin-Ouellet J. Mitochondrial complex I deficiency and Parkinson disease. Nat Rev Neurosci. 2023;244:193. Article Google Scholar Schapira AH, Mann VM, Cooper JM, Krige D, ...
摘要: Background The Coq protein complex assembled from several Coq proteins is critical for coenzyme Q6 关键词: High-molecular-weight protein complex Human COQ5 Human COQ9 Chemical uncoupler mtDNA mutation Secondary coenzyme \{Q10\} deficiency ...
5 Evidence for a mitochondrial-related cause in PD stems from studies reporting on the use of human postmortem brains of patients with PD, which found a deficiency of complex I of the mitochondrial respiratory chain in the SNpc.6 Furthermore, outside the central nervous system a mitochondrial ...
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 ...
identified that the deficiency of nuclear-encoded mitochondrial inorganic pyrophosphatase (PPA2) due to biallelic missense mutations was associated with cardiac fibrosis by using whole-exome sequencing, of which mechanism is related to suppress the activity of respiratory chain complex I and IV, and ...
A complex network of interactions between longevity pathways reveals an intricate regulation of mitochondrial physiology during ageing. Cellular metabolism interconnects the nine hallmarks of ageing, and deregulation of energy metabolism by environmental variations is an essential process leading to mitochondrial...