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1 ctron-accepting complex of the mitochondrial respiratory chain.
2 l, which is generated by the activity of the respiratory chain.
3 slation, or a component of the mitochondrial respiratory chain.
4 their putative positions in the aerobic iron respiratory chain.
5 sential to the function of the mitochondrial respiratory chain.
6 en species, a byproduct of the mitochondrial respiratory chain.
7 hemoglobin, myoglobin, and components of the respiratory chain.
8 t nematocidal activity via the mitochondrial respiratory chain.
9 a multisubunit, membrane-bound enzyme of the respiratory chain.
10 eases due to a biochemical deficiency of the respiratory chain.
11 ed glutamine metabolism and Complex 1 of the respiratory chain.
12 a role in the assembly of the mitochondrial respiratory chain.
13 nes encoding components of the mitochondrial respiratory chain.
14 translocase-associated import motor and the respiratory chain.
15 including the complex I (CI) subunits in the respiratory chain.
16 he operation of the protein complex I of the respiratory chain.
17 plex with multiple components of the aerobic respiratory chain.
18 entry point for electrons from NADH into the respiratory chain.
19 through oxidative phosphorylation within the respiratory chain.
20 y evolving genome and lacks complex I of the respiratory chain.
21 and functions as a negative regulator of the respiratory chain.
22 de, confirming the oxidation occurred in the respiratory chain.
23 to an important role for the function of the respiratory chain.
24 y of all four complexes of the mitochondrial respiratory chain.
25 ydrogen peroxide (H(2)O(2)) generated by the respiratory chain.
26 taPsi(m)) generated by proton pumping by the respiratory chain.
27 nslocase of the outer membrane (TOM) and the respiratory chain.
28 which encode components of the mitochondrial respiratory chain.
29 e (CcO) is the last electron acceptor in the respiratory chain.
30 energy disruptor targeting complex I of the respiratory chain.
31 ighly potent inhibitors of the mitochondrial respiratory chain.
32 hat GLDH is associated with complex I of the respiratory chain.
33 idation, tricarboxylic acid (TCA) cycle, and respiratory chain.
34 totrophs but can also be involved in aerobic respiratory chains.
35 ich is reflected in the composition of their respiratory chains.
36 y point into the mitochondrial and bacterial respiratory chains.
37 tase, and FeS cluster-containing subunits of respiratory chains.
38 ons as a redox-driven proton pump in aerobic respiratory chains.
39 despread CoQ(9) deficiency and mitochondrial respiratory chain abnormalities, only affected organs sh
41 reased ATP synthesis, oxygen consumption and respiratory chain activities compared to cybrids with ca
42 sue showed reduced mitochondrial density and respiratory chain activity along with increased mitophag
45 ated activation of matrix dehydrogenases and respiratory chain activity by calcium allows the respira
47 ions, and their ablation results in impaired respiratory chain activity, increased oxidative stress,
48 resulting flies are characterized by lowered respiratory chain activity, premature aging, age-related
50 s have a lower spare reserve capacity in the respiratory chain and are more susceptible to oxidative
51 oduction of hydrogen peroxide was a block in respiratory chain and diversion of electrons from NADH o
52 lex I is the first and largest enzyme of the respiratory chain and has a central role in cellular ene
53 efects in all complexes of the mitochondrial respiratory chain and in the F-ATP synthase, while adult
56 s activity is linked to the operation of the respiratory chain and is essential for the development o
57 ion will expand our understanding of how the respiratory chain and mitochondrial ROS influence whole
58 work underscores the connection between the respiratory chain and one-carbon metabolism with implica
60 T mice presented a similar impairment of the respiratory chain and phosphorylation system, decreased
61 esigned to directly target the mitochondrial respiratory chain and prevent excessive reactive oxygen
62 ed pathway essential for the function of the respiratory chain and several mitochondrial enzyme compl
63 ncluding key metabolic pathways, such as the respiratory chain and the photosystems, as well as the t
65 olamine are required for the activity of the respiratory chain and therefore to maintain the membrane
66 bunits of the NADH:quinone oxidoreductase of respiratory chains and to subunits of several hydrogenas
67 mately maintained by the proton pumps of the respiratory chain, and Ca(2+) binding to matrix buffers.
68 s predicted to be important in thaumarchaeal respiratory chain, and the absence of a high-affinity ph
69 etabolism; a partial Kreb's cycle; a reduced respiratory chain; and a laterally acquired rhodoquinone
70 observation that cells lacking a functional respiratory chain are auxotrophic for pyruvate, which se
71 mutants in nuclear-encoded components of the respiratory chain are non-viable, emphasizing the import
72 he physical arrangement of the mitochondrial respiratory chain as a compact physical entity (the soli
73 of increasing the electron flux through the respiratory chain as a strategy to induce oxidative stre
74 cially highlighting lipid metabolism and the respiratory chain as important pathways involved in neur
75 proposed in another study, we identified the respiratory chain as the most probable target of Hint2.
76 n contrast, the inhibition of the chlamydial respiratory chain at mid-stage of the infection cycle de
77 disrupting the flow of electrons through the respiratory chain at the cytochrome bc1 complex, causing
78 is the most commonly observed mitochondrial respiratory chain biochemical defect, affecting the larg
81 2 and Rcf3 increases oxygen flux through the respiratory chain by up-regulation of the cytochrome c o
82 ll and exemplify how the plant mitochondrial respiratory chain can act as a multifunctional electron
83 piration and calcium retention capacity, and respiratory chain complex activities and oxidative stres
84 d droplets had decreased in size and number, respiratory chain complex activities were partially rest
88 All had biochemical evidence of multiple respiratory chain complex defects but no primary pathoge
90 out of nine families presented with combined respiratory chain complex deficiencies in skeletal muscl
92 AD9) is an assembly factor for mitochondrial respiratory chain Complex I (CI), and ACAD9 mutations ar
94 750]), revealed both proteins are present in respiratory chain complex I and the Translocase of the I
97 und to have profoundly decreased activity of respiratory chain complex I in muscle, heart and liver.
98 f chronic low-dose rotenone, a mitochondrial respiratory chain complex I inhibitor, to alter lithium-
100 ciates with the oncoprotein survivin and the respiratory chain Complex II subunit succinate dehydroge
102 of the preferential interfaces of CLs on the respiratory chain complex III (cytochrome bc(1), CIII).
105 specific biochemical defect of mitochondrial respiratory chain complex III, and explores the impact o
106 cts in mitochondrial cytochrome c oxidase or respiratory chain complex IV (CIV) assembly are a freque
107 ic decrease in the activity of mitochondrial respiratory chain complex IV (cytochrome c oxidase, COX)
109 duction of mitochondrial DNA copy number and respiratory chain complex IV levels, altered mitochondri
110 ndrial function, in particular mitochondrial respiratory chain complex IV or cytochrome c oxidase act
111 rol cells undergoing normal respiration, the respiratory chain complex IV subunit 1 (COX1) was tightl
112 f cytochrome c oxidase (MTCO1), a subunit of respiratory chain complex IV, mitochondrial transcriptio
113 localized in mitochondria and interacts with respiratory chain complex IV, suggesting that Mb could b
114 and the general depression of mitochondrial respiratory chain complex levels (including complex II)
115 dosis and evidence of multiple mitochondrial respiratory-chain-complex deficiencies in skeletal muscl
116 The specific type of brain mitochondrial respiratory chain complexes (mRCC) that are adversely af
117 mbalanced stoichiometry of the mitochondrial respiratory chain complexes and skin inflammation and su
118 decrease of AAC1 protein levels and loss of respiratory chain complexes containing mitochondrial DNA
121 hondrial biochemical abnormalities involving respiratory chain complexes I and IV due to clonally-exp
122 rasomes are macromolecular assemblies of the respiratory chain complexes I, III and IV in the inner m
125 creases in transcript polyadenylation and in respiratory chain complexes were effectively rescued by
126 d TCA cycle enzymes as well as components of respiratory chain complexes were selected and assessed f
127 ain how AIF contributes to the biogenesis of respiratory chain complexes, and they establish an unexp
128 to downregulated expression and activity of respiratory chain complexes, features characteristic of
129 an imbalanced stoichiometry of mitochondrial respiratory chain complexes, inducing a unique combinati
130 s, decreased activities of the mitochondrial respiratory chain complexes, multiple mitochondrial DNA
131 h impaired activity of various mitochondrial respiratory chain complexes, were observed in muscle bio
139 horylation system, which is composed of four respiratory-chain complexes and adenosine triphosphate (
142 nuclear and mitochondrial genes that encode respiratory chain components and by NOTCH-dependent indu
143 s an opportunity to determine how individual respiratory chain components contribute to physiology fo
144 ately quantify the presence of mitochondrial respiratory chain components within individual bone cell
147 The genomic data suggest that C. trachomatis respiratory chain could produce a sodium gradient that m
148 Inhibition of the function of classical respiratory chain (CRC) led to a decrease in the electro
150 phenformin, an inhibitor of complex I of the respiratory chain, decreased the oxygen consumption rate
151 in glucose-free galactose medium revealed a respiratory chain defect in complexes I and II, and a tr
152 tion of mitochondrial DNA (mtDNA) damage and respiratory chain defect, metabolic disturbance, pro-apo
153 Furthermore, the toxicity of DEHP led to respiratory chain defects and attenuation of ATP level p
154 ical presentation of mitochondrial diseases, respiratory chain defects and defects of complex I speci
155 he regulatory mechanisms involved in sensing respiratory chain defects and modifying mitochondrial fu
156 g pathway in which DOX-induced mitochondrial respiratory chain defects and necrotic cell death are mu
157 thway that couples DOX-induced mitochondrial respiratory chain defects and necrotic cell death to the
158 ine in tissues, mitochondrial DNA depletion, respiratory chain defects and white matter changes.
159 Altogether, our results suggest that partial respiratory chain defects because of mtDNA mutations can
161 (COX) deficiency is one of the most frequent respiratory chain defects seen in human mitochondrial di
162 feature of mitochondrial disorders caused by respiratory chain defects, notably, cytochrome oxidase I
166 ctor 21, a novel biomarker for mitochondrial respiratory chain deficiencies and inhibitor of white ad
167 sted, revealed severe combined mitochondrial respiratory chain deficiencies associated with a marked
168 ve useful as a therapeutic tool for limiting respiratory chain deficiencies caused by mtDNA decline i
171 rome c oxidase-negative fibres with combined respiratory chain deficiency and abnormal assembly of co
172 e, while somatic mtDNA deletions cause focal respiratory chain deficiency associated with ageing and
173 teins in passively cleared tissues to reveal respiratory chain deficiency associated with mitochondri
176 red mitochondrial biogenesis and progressive respiratory chain deficiency were also evident in cardio
177 mutations in this protein result in a severe respiratory chain deficiency, loss of mitochondrial memb
180 s group of metabolic disorders with combined respiratory-chain deficiency and a neuromuscular phenoty
181 hput screen (HTS) was undertaken against the respiratory chain dehydrogenase component, NADH:menaquin
182 imaging reveals that contractions constitute respiratory chain-dependent episodes of depolarization c
183 one oxidoreductase), the first enzyme of the respiratory chain, display various phenotypes depending
184 also produces coenzyme Q (a component of the respiratory chain), dolichols (important for protein gly
185 atic mitochondrial DNA (mtDNA) mutations and respiratory chain dysfunction accompany normal aging, bu
188 ide two lines of evidence that mitochondrial respiratory chain dysfunction leads to alterations in on
189 nd skin inflammation and suggest that severe respiratory chain dysfunction, as observed in few cells
190 accumulates within mitochondria, but during respiratory chain dysfunction, high levels of reactive o
191 drial abnormalities, including mitochondrial respiratory chain dysfunction, reduced ATP synthesis and
192 AD synthase (FADS), as the cause of MADD and respiratory-chain dysfunction in nine individuals recrui
193 or succinate with different sections of the respiratory chain engaged in catalysis as a proxy for th
195 s and differentiated cells had mitochondrial respiratory chain enzyme activities and oxygen consumpti
196 required for optimal TRMU function, rescued respiratory chain enzyme activities in human cell lines
198 r investigations including muscle biopsy and respiratory chain enzyme activity were non-specific or n
200 ll individuals' samples, leading to combined respiratory-chain enzyme deficiency of complexes I, III,
201 tein interactions regulate the activity of a respiratory-chain enzyme, CymA, by changing the directio
203 mutants and revealed that deleting genes for respiratory chain flavoproteins or for tricarboxylic aci
204 lobacter jejuni uses complex cytochrome-rich respiratory chains for growth and host colonisation.
206 into host respiratory complexes, decreasing respiratory chain function and increasing oxidative stre
208 radiation-induced mitochondrial fission, but respiratory chain function in mitochondria inhibited by
210 mitochondrial distribution or mitochondrial respiratory chain function result in corresponding chang
211 NNT)], we selectively impaired mitochondrial respiratory chain function, energy exchange, and mitocho
216 other stress-resistant genes, mitochondrial respiratory chain genes, and potential IIS receptor anta
217 oxidases or Complex III of the mitochondrial respiratory chain, H2O2 is under sophisticated fine cont
218 itor of the mitochondrial and photosynthetic respiratory chain, has been proven to be a unique voltag
221 ficiency is the most prevalent defect in the respiratory chain in paediatric mitochondrial disease.
222 d thus target complexes of the mitochondrial respiratory chain in the inner mitochondrial membrane.
224 formate from serine, and that in some cells, respiratory chain inhibition leads to growth defects upo
227 le human cancer cells, whereas mitochondrial respiratory chain inhibitors do not affect CHCM1/CHCHD6
228 roton-pumping complex I of the mitochondrial respiratory chain is among the largest and most complica
231 O), the terminal enzyme of the mitochondrial respiratory chain, is a complex process facilitated by s
232 was caused by inhibition of Complex I of the respiratory chain, leading to increases in cellular AMP
233 issues and species responsive to H2O2 as the respiratory chain, Lyn, and Syk were similarly required
234 ncomitant up-regulation of the mitochondrial respiratory chain (MRC) activity induces reactive oxygen
235 nfluencing the assembly of the mitochondrial respiratory chain (MRC) complexes into supercomplexes.
238 xygen-dependent oxidation of the multicenter respiratory chain occurred with a single macroscopic rat
239 tron transport (EET) pathway for linking the respiratory chain of bacteria to external surfaces, incl
240 genase (Na(+)-NQR) is a key component of the respiratory chain of diverse prokaryotic species, includ
242 reductase, bc1 complex, is the enzyme in the respiratory chain of mitochondria responsible for the tr
243 venging serves as a mechanism to sustain the respiratory chain of P. methylaliphatogenes when organic
244 oxs) are the basic energy transducers in the respiratory chain of the majority of aerobic organisms.
247 nctions as a redox-linked proton pump in the respiratory chains of mitochondria and bacteria, driven
248 pe cytochrome c oxidase (CcO) terminates the respiratory chains of mitochondria and many bacteria.
251 ium nitroprusside (complex inhibitors of the respiratory chain) on mitochondrial function were also s
252 which required increased supply of NADH for respiratory chain oxidoreductases from central carbon ca
253 ase (COX) or complex IV of the mitochondrial respiratory chain plays a fundamental role in energy pro
254 X), the terminal enzyme of the mitochondrial respiratory chain, plays a key role in regulating mitoch
255 t require heme for electron transport in the respiratory chain, protection against oxidative stress,
256 found that ClpP interacts with mitochondrial respiratory chain proteins and metabolic enzymes, and kn
257 stages of prion infection, dysregulation of respiratory chain proteins may lead to impairment of mit
260 lactic acidemia in association with combined respiratory chain (RC) deficiencies of complexes I, III
265 contrast, the turnover of many mitochondrial respiratory chain (RC) subunits showed greater impairmen
267 nction was ameliorated and the mitochondrial respiratory chain recovered following obeticholic acid t
268 vely, our data lend mechanistic insight into respiratory chain-related activities and prioritize hund
269 ng several key proteins in the mitochondrial respiratory chain (rho(0) cells), the membrane potential
270 ce, which are deficient in the mitochondrial respiratory chain subunit Ndufs4 [NADH dehydrogenase (ub
271 c translational activators ensure that these respiratory chain subunits are synthesized at the correc
272 regulates the expression of nuclear-encoded respiratory chain subunits involved in Complexes I, II,
273 technique enabling the quantification of key respiratory chain subunits of complexes I and IV, togeth
275 analysis confirmed the reduced levels of the respiratory chain subunits that included mitochondrially
277 but do not preserve mitochondrial cristae or respiratory chain supercomplex assembly in prohibitin-de
281 l facilitates the formation of mitochondrial respiratory chain supercomplexes to sustain high mitocho
283 Finally, we discuss how the formation of respiratory-chain supercomplexes may confer alternative
285 O), the terminal enzyme in the mitochondrial respiratory chain that catalyzes the reduction of oxygen
286 cing of ND75, a subunit of the mitochondrial respiratory chain that controls ROS formation independen
287 predictions of the behavior of the mammalian respiratory chain that depend on whether channeling in s
288 heir essential role in the biogenesis of the respiratory chain, the molecular function of twin Cx9C p
289 rmembrane space, delivering electrons to the respiratory chain through CYTc These results provide a c
290 a signal transduction pathway that links the respiratory chain to the mitochondrial intermembrane spa
291 of complex I is formed during hypoxia, when respiratory chain turnover is slowed, and may contribute
292 esentative PRC-dependent stress genes by the respiratory chain uncoupler, carbonyl cyanide m-chloroph
293 ponse as the results place the mitochondrial respiratory chain upstream of tyrosine-protein kinase Ly
294 nts from cytosolic NADH to the mitochondrial respiratory chain via the D-lactate dehydrogenase Dld1.
295 ogenesis, and Cox-1, a critical component of respiratory chain, was significantly increased in M2 pol
296 rthermore, by compromising components of the respiratory chain, we demonstrated that the reliance on
297 processes such as photosynthesis and in the respiratory chain, where they mediate long-range charge
298 s succinate:quinone reductase as part of its respiratory chain, whereas under microaerophilic conditi
299 ssion of the complex IV of the mitochondrial respiratory chain), which is compromised in NPC1 cells.
300 It is the largest protein assembly of the respiratory chain with a total mass of 970 kilodaltons.
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