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Alvarez-Mora 2016 Mol Neurobiol
Additional label Labels  + , 2018-10  +
Coupling states ROUTINE  +
Diseases Neurodegenerative  +
Has abstract Mitochondrial involvement plays an importa
Mitochondrial involvement plays an important role in neurodegenerative diseases. At least one-third of adult carriers of a ''FMR1'' premutation (55-200 CGG repeats) are at risk of presenting an adult-onset neurodegenerative disorder known as fragile X-associated tremor/ataxia syndrome (FXTAS). In an attempt to provide new insights into the mechanisms involved in the pathogenesis of FXTAS, we characterized mitochondrial function and dynamics by the assessment of oxidative respiratory chain function, mitochondrial content, oxidative stress levels, and mitochondrial network complexity. Regarding mitochondrial function, we found that mitochondrial respiratory capacity is compromised in skin fibroblasts whereas in blood, no differences were observed between the FXTAS and control groups. Furthermore, fibroblasts from FXTAS patients presented altered mitochondrial architecture, with more circular and less interconnected mitochondria being observed. Mitochondrial function and dynamics deregulation and characteristic of neurological disorders are present in FXTAS patients. These features might be limiting temporal and spatial bioenergetics cells supply and thus contributing to disease pathogenesis.
thus contributing to disease pathogenesis.  +
Has editor [[Plangger M]]  +
Has info [https://www.ncbi.nlm.nih.gov/pubmed/27771901 PMID: 27771901]  +
Has publicationkeywords FXTAS  + , Mitochondrial dynamics  + , Mitochondrial dysfunction  + , Oxygen uptake  + , ROS production  +
Has title Alvarez-Mora MI, Rodriguez-Revenga L, Madrigal I, Guitart-Mampel M, Garrabou G, Milà M (2016) Impaired mitochondrial function and dynamics in the pathogenesis of FXTAS. Mol Neurobiol 54:6896-902.  +
Instrument and method Oxygraph-2k  +
Mammal and model Human  +
MiP area Respiration  +
Pathways N  + , S  + , NS  +
Preparation Intact cells  +
Tissue and cell Fibroblast  +
Was published by MiPNetLab ES Barcelona Moren C +
Was published in journal Mol Neurobiol +
Was published in year 2016  +
Was written by Alvarez-Mora MI + , Rodriguez-Revenga L + , Madrigal I + , Guitart-Mampel M + , Garrabou G + , Mila M +
Categories Publications
Modification date
"Modification date" is a predefined property that corresponds to the date of the last modification of a subject and is provided by Semantic MediaWiki.
12:10:21, 4 October 2018  +
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