The mitochondrial
respiratory chain is one of the most important endogenous sources in the production of reactive oxygen species (ROS).
Antibodies against subunits of the
respiratory chain are markers of mitochondrial hyperplasia.
Considering our data and the studies on biochemical evaluation of
respiratory chain activities, single fiber analysis and steady state of the G5835A MT-TY variant performed by Kornblum et al.
Abbreviations BSCL2: Berardinelli-Seip congenital lipodystrophy CMT2D: Charcot-Marie-Tooth 2D DCTN1: Dynactin subunit 1 dHMN: Distal hereditary motor neuropathies dSMA-V: Distal spinal muscular atrophy type V dSMA-Va: Distal spinal muscular atrophy type Va EMG: Electromyography ETC: Mitochondrial
respiratory chain enzyme analysis GARS: Glycyl tRNA synthetase IGHMBP2: Immunoglobulin [mu]-binding protein-2 L: Leucine R: Arginine SETX: Senataxin sHSP: Small heat shock proteins SMA: Spinal muscular atrophy RSV: Respiratory syncytial virus VUS: Variant of unknown significance WES: Whole exome sequencing.
oleifera protective effect, we measured the mitochondrial
respiratory chain using Clark-type oxygen electrode and determined enzymatic activity of each complex by spectrophotometric methods.
This makes it possible to determine the efficiency of the individual complexes within the
respiratory chain. Oligomycin blocks the [F.sub.0] subunit of the ATP synthase.
Importantly, the exposure to toxins like the pesticide rotenone and the herbicide paraquat reduced the activity of mitochondrial
respiratory chain complex I and caused neurological defects similar to PD in humans and in animal models [37, 38].
The transcripts of some nuclear-encoded proteins from the mitochondrial
respiratory chain of the shrimp Litopenaeus vannamei are known to be downregulated by hypoxia, including some proteins that compose the Frcatalytic domain of the ATP synthase (Martinez-Cruz et al.
Electron leakage in
respiratory chain serves as the main source for generating reactive oxygen species and free radicals (superoxide anion radical [O.sub.2.sup.-*], hydrogen peroxide ([H.sub.2]O2) molecules produced by superoxide dismutase).
It codes 22 tRNA, 2 rRNA and 13 necessary subunits of the
respiratory chain complexes (18, 19).
Metformin works by lowering blood glucose and also blocks the
respiratory chain in the cells' mitochondria (See What You Should Know), while the drug syrosingopine inhibits degradation of sugars, interrupting processes that provide energy for the cells.
Several dehydrogenases transfer electrons to ETF, which transfer to ETF-QO and ultimately to coenzyme Q within the
respiratory chain. MADD is classified into 3 groups based on clinical phenotype: (a) neonatal form with congenital anomalies; (b) neonatal form without anomalies; and (c) mild form characterized by ethylmalonicadipic aciduria.
