Under metabolic stress, several ionic channels present in the inner and outer mitochondrial membranes can have pro-life and -death effects. [books.google.de]

Expressions of caspase-1, caspase-2, caspase-3, caspase-7, and caspase-8 were increased while expression of Bcl-2 was decreased in cells expressing mutated Htt-exon 1. (4) Results presented in this communication showed that expression of mutated Htt-exon [ncbi.nlm.nih.gov]

At present, we can only speculate about the selective advantage that SDH2.3 may confer to complex II. [frontiersin.org]

We describe a young woman presenting an early-onset, progressive neurodegenerative disorder with decreased mitochondrial complex I and IV activities in skeletal muscle. [ng.neurology.org]

Previous Presentations: This study was presented in part at the 64th Annual Convention of the Society of Biological Psychiatry; May 14, 2009; Vancouver, British Columbia, Canada, and at the 63rd Annual Convention of the Society of Biological Psychiatry [jamanetwork.com]

• Inflammation

  The specific proposed sections are: 1) Neuroimmunity and Neuroinflammation; 2) Signalling and inflammation in aging; 3) Neurodegeneration and inflammation in age related diseases. [books.google.de]

• Collapse

  This, in turn, leads to a simultaneous collapse of mitochondrial membrane potential and the activation of selective elimination of depolarized and dysfunctional mitochondria by mitophagy. [books.google.de]

• Movement Disorder

  Together with Professor Stanley Fahn, of the Neurological Institute in New York, he founded the Movement Disorder Society and its scientific journal Movement Disorders. He died in 1998. [books.google.de]

• Dystonia

  His main research interest is in movement disorders, specifically the merging of clinical, electrophysiological and imaging methods to provide insights into the pathophysiology of conditions like dystonia and Parkinson's disease. [books.google.de]

Methods: A case report including diagnostic workup, whole-exome sequencing of the affected patient, filtering, and prioritization of candidate variants assuming a suspected autosomal recessive mitochondrial disorder and segregation studies. [ng.neurology.org]

“Our research means that we now have a specific biomarker, or a target, for developing and screening anti-ageing treatments and cosmetic creams that may counter this decline in bio-energy. [telegraph.co.uk]

treatments and cosmetic products that could counteract this decrease in mitochondrial complex II in aging human skin, and treatment could even be “tailored to differently aged and differently pigmented skin.” [kurzweilai.net]

With these results, scientists can develop more treatments for age-related diseases, such as cancer, with the understanding of how other body organs age. [revivserums.com]

Global gene-expression analysis revealed that there was 100-fold more genes differentially expressed between dsr1 and WT upon SA treatment than in the mock treatment ( Fig. S3 ). [pnas.org]

Multiple deletion Ataxia, Hypogonadism & Choroidal dystrophy: Multiple deletion Isolated Complex I deficit: Nuclear mutations Clinical features: General Nuclear mutations more common than mitochondrial Onset: Childhood; Mean 4 to 5 months Male:Female 3.5:1 Prognosis [neuromuscular.wustl.edu]

Taylor First published September 22, 2017, DOI: Abstract Objective: To determine the genetic etiology of a young woman presenting an early-onset, progressive neurodegenerative disorder with evidence of decreased mitochondrial complex I and IV activities [ng.neurology.org]

Despite decades of extensive investigation, the etiology and pathogenesis of this disorder remain unclear. [jamanetwork.com]

Epidemiology and treatment of mitochondrial disorders. Am J Med Genet 2001 ; 106 : 94 -101. Valnot I, Kassis J, Chretien D, de Lonlay P, Parfait B, Munnich A, et al. [clinchem.aaccjnls.org]

His main research interest is in movement disorders, specifically the merging of clinical, electrophysiological and imaging methods to provide insights into the pathophysiology of conditions like dystonia and Parkinson's disease. [books.google.de]

Succinate supplementation prevented decreases in complex I activity. ATP levels decreased 43% in injured RPTC but were maintained in injured cells supplemented with succinate. [jpet.aspetjournals.org]

[…] des maladies mitochondriales Urtizberea JA 2018 Myopathie mitochondriale : la piste de la thérapie génique Cukierman L 2018 Myopathie mitochondriale : résultats positifs d’un essai clinique de la taurine Cukierman L 2018 Taurine supplementation for prevention [myobase.org]

Its role is to prevent the interaction of the intermediate with molecular oxygen to produce reactive oxygen species (ROS). The heme group, relative to ubiquinone, is shown in image 4. [en.wikipedia.org]

For a fungal pathogen like R. solani AG8, which is unable to penetrate the roots of WT plants ( 16 ), mROS could contribute to the oxidative cross-linking of the cell wall to help prevent fungal penetration ( 31, 32 ). [pnas.org]

And second, bZIP53 bZIP10 activate the SDH2.3 promoter in leaf protoplasts, and this activation is reduced when the ABRE2 cis -element is mutated to prevent transcription factor binding (Figure 3 ). [frontiersin.org]