نتایج جستجو برای: mitochondrial respiratory chain complex i

تعداد نتایج: 2265450  

2011
Thorsten Althoff Deryck J Mills Jean-Luc Popot Werner Kühlbrandt

The respiratory chain in the inner mitochondrial membrane contains three large multi-enzyme complexes that together establish the proton gradient for ATP synthesis, and assemble into a supercomplex. A 19-Å 3D map of the 1.7-MDa amphipol-solubilized supercomplex I(1)III(2)IV(1) from bovine heart obtained by single-particle electron cryo-microscopy reveals an amphipol belt replacing the membrane ...

Journal: :The Journal of clinical investigation 2004
Denise M Kirby Renato Salemi Canny Sugiana Akira Ohtake Lee Parry Katrina M Bell Edwin P Kirk Avihu Boneh Robert W Taylor Hans-Henrik M Dahl Michael T Ryan David R Thorburn

complex I deficiency, the most common respiratory chain defect, is genetically heterogeneous: mutations in 8 nuclear and 7 mitochondrial DNA genes encoding complex I subunits have been described. However, these genes account for disease in only a minority of complex I-deficient patients. We investigated whether there may be an unknown common gene by performing functional complementation analysi...

Journal: :Mitochondrion 2014
Simon Massoz Véronique Larosa Charlotte Plancke Marie Lapaille Benjamin Bailleul Dorothée Pirotte Michèle Radoux Pierre Leprince Nadine Coosemans René F Matagne Claire Remacle Pierre Cardol

In Chlamydomonas, unlike in flowering plants, genes coding for Nd7 (NAD7/49 kDa) and Nd9 (NAD9/30 kDa) core subunits of mitochondrial respiratory-chain complex I are nucleus-encoded. Both genes possess all the features that facilitate their expression and proper import of the polypeptides in mitochondria. By inactivating their expression by RNA interference or insertional mutagenesis, we show t...

2012
Michael Erb Barbara Hoffmann-Enger Holger Deppe Michael Soeberdt Roman H. Haefeli Christian Rummey Achim Feurer Nuri Gueven

Short-chain quinones have been investigated as therapeutic molecules due to their ability to modulate cellular redox reactions, mitochondrial electron transfer and oxidative stress, which are pathologically altered in many mitochondrial and neuromuscular disorders. Recently, we and others described that certain short-chain quinones are able to bypass a deficiency in complex I by shuttling elect...

Journal: :Biochemical Society transactions 1995
S R Hawtin A C Dobbins V J Tailor M S Shearman

Senile plaques, a neuropathological feature of Alzheimer's disease, consist mainly of insoluble aggregates of pamyloid protein I I I . Using rat pheochromocytoma PC12 cells as an irr vitro model. i t has been shown that 8-amyloid potently inhibits the reduction of MTT 12.31. The cellular reduction of M 7 T is thought to be mediated by the mitochondrial respiratory chain, although other NAD(P)H ...

Journal: :Kidney international 2011
Aihua Zhang Zhanjun Jia Ningning Wang Tyson J Tidwell Tianxin Yang

We assessed the relative contribution of the mitochondrial respiratory chain and NADPH (nicotinamide adenine dinucleotide phosphate) oxidase to deoxycorticosterone acetate (DOCA)-salt hypertension in mice. The daily mean arterial pressure was monitored by radiotelemetry in DOCA-salt-treated mice given vehicle or the mitochondrial respiratory chain complex I inhibitor rotenone. This treatment pr...

2010
Andrey Y. Abramov Tora K. Smulders-Srinivasan Denise M. Kirby Rebeca Acin-Perez José Antonio Enriquez Robert N. Lightowlers Michael R. Duchen Douglass M. Turnbull

Mutations of mitochondrial DNA are associated with a wide spectrum of disorders, primarily affecting the central nervous system and muscle function. The specific consequences of mitochondrial DNA mutations for neuronal pathophysiology are not understood. In order to explore the impact of mitochondrial mutations on neuronal biochemistry and physiology, we have used fluorescence imaging technique...

2014
Federica Invernizzi Anna Ardissone Eleonora Lamantea Barbara Garavaglia Massimo Zeviani Laura Farina Daniele Ghezzi Isabella Moroni

Multiple Mitochondrial Dysfunction Syndrome (MMDS) comprises a group of severe autosomal recessive diseases with onset in early infancy and characterized by a systemic disorder of energy metabolism, resulting in weakness, respiratory failure, lack of neurological development, lactic acidosis, and early death. Biochemical findings include defects of complexes I, II, and III of the mitochondrial ...

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