نتایج جستجو برای: mitochondrial respiratory chain complex i

تعداد نتایج: 2265450  

Journal: :Physiological research 2017
M Urbanová M Mráz V Ďurovcová P Trachta J Kloučková P Kaválková D Haluzíková Z Lacinová H Hansíková L Wenchich M Kršek M Haluzík

Mitochondrial dysfunction is a potentially important player in the development of insulin resistance and type 2 diabetes mellitus (T2DM). We investigated the changes of mRNA expression of genes encoding main enzymatic complexes of mitochondrial respiratory chain in subcutaneous adipose tissue (SCAT) and peripheral monocytes (PM) of 11 subjects with simple obesity (OB), 16 obese patients with T2...

Journal: :The Journal of biological chemistry 2005
Tiesong Shang Joy Joseph Cecilia J Hillard B Kalyanaraman

We have investigated here the mechanism of dephosphorylation and activation of death-associated protein kinase (DAPK) and the role of lysosome in neuroblastoma cells (SH-SY5Y) treated with mitochondrial toxins, such as MPP(+) and rotenone. Mitochondrial respiratory chain inhibitors and uncouplers decreased mitochondrial membrane potential leading to DAPK dephosphorylation and activation. The cl...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2005
Leslie I Grad Leanne C Sayles Bernard D Lemire

Mitochondrial dysfunction, with an estimated incidence of 1 in 5,000 births, is associated with a wide variety of multisystem degenerative diseases. Among the most prevalent forms of dysfunction are defects in the NADH:ubiquinone oxidoreductase (complex I). Caenorhabditis elegans strains with complex I mutations exhibit characteristic features of human mitochondrial disease including decreased ...

Journal: :Cureus 2023

A 74-year-old woman with type 2 diabetes mellitus developed ketoacidosis within six days of adding metformin to imeglimin treatment. The patient was insulin-sensitive and showed preserved insulin secretion; therefore, insufficiency alone unlikely contribute the development ketoacidosis. Both partially inhibit complex I in mitochondrial respiratory chain. Inhibition respiration can lead tricarbo...

Journal: :Sains Malaysiana 2022

Mitochondrial dysfunction is common in the brain with age. Prevention of mitochondrial at an early age may protect against neurodegeneration later life. Tocotrienol-rich fraction (TRF) has been reported to be neuroprotective old rats, but its effect remains unknown for middle-aged animals. This study aimed determine TRF on activities respiratory chain complexes hippocampus rats. Male Sprague Da...

Journal: :American journal of human genetics 2008
Canny Sugiana David J Pagliarini Matthew McKenzie Denise M Kirby Renato Salemi Khaled K Abu-Amero Hans-Henrik M Dahl Wendy M Hutchison Katherine A Vascotto Stacey M Smith Robert F Newbold John Christodoulou Sarah Calvo Vamsi K Mootha Michael T Ryan David R Thorburn

Complex I (NADH:ubiquinone oxidoreductase) is the first and largest multimeric complex of the mitochondrial respiratory chain. Human complex I comprises seven subunits encoded by mitochondrial DNA and 38 nuclear-encoded subunits that are assembled together in a process that is only partially understood. To date, mutations causing complex I deficiency have been described in all 14 core subunits,...

Journal: :Biochimica et biophysica acta 2017
Samuel Frey Guillaume Geffroy Valerie Desquiret-Dumas Naig Gueguen Celine Bris Sophie Belal Patrizia Amati-Bonneau Arnaud Chevrollier Magalie Barth Daniel Henrion Guy Lenaers Dominique Bonneau Pascal Reynier Vincent Procaccio

Ketogenic Diet used to treat refractory epilepsy for almost a century may represent a treatment option for mitochondrial disorders for which effective treatments are still lacking. Mitochondrial complex I deficiencies are involved in a broad spectrum of inherited diseases including Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-like episodes syndrome leading to recurrent cerebral i...

Journal: :American journal of physiology. Regulatory, integrative and comparative physiology 2012
Miri Assayag Ann Saada Gary Gerstenblith Haifa Canaana Rivka Shlomai Michal Horowitz

Long-term heat acclimation (LTHA; 30 days, 34°C) causes phenotypic adaptations that render protection against ischemic/reperfusion insult (I/R, 30 min global ischemia and 40 min reperfusion) via heat acclimation-mediated cross-tolerance (HACT) mechanisms. Short-term acclimation (STHA, 2 days, 34 °C), in contrast, is characterized by cellular perturbations, leading to increased susceptibility to...

2017
Andreea Manole Zane Jaunmuktane Iain Hargreaves Marthe H R Ludtmann Vincenzo Salpietro Oscar D Bello Simon Pope Amelie Pandraud Alejandro Horga Renata S Scalco Abi Li Balasubramaniem Ashokkumar Charles M Lourenço Simon Heales Rita Horvath Patrick F Chinnery Camilo Toro Andrew B Singleton Thomas S Jacques Andrey Y Abramov Francesco Muntoni Michael G Hanna Mary M Reilly Tamas Revesz Dimitri M Kullmann James E C Jepson Henry Houlden

Brown-Vialetto-Van Laere syndrome represents a phenotypic spectrum of motor, sensory, and cranial nerve neuropathy, often with ataxia, optic atrophy and respiratory problems leading to ventilator-dependence. Loss-of-function mutations in two riboflavin transporter genes, SLC52A2 and SLC52A3, have recently been linked to Brown-Vialetto-Van Laere syndrome. However, the genetic frequency, neuropat...

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