%0 Journal Article %T Energy defects in Huntington's disease: Why “in vivo” evidence matters %+ Institut d'Imagerie BioMédicale (I2BM) %+ Laboratoire des Maladies Neurodégénératives - UMR 9199 (LMN) %+ Commissariat à l'énergie atomique et aux énergies alternatives (CEA) %+ Inserm U967, Fontenay‐aux‐Roses cedex, France %+ Service MIRCEN (MIRCEN) %A Liot, Géraldine %A Valette, Julien %A Pépin, Jérémy %A Flament, Julien %A Brouillet, Emmanuel %< avec comité de lecture %@ 0006-291X %J Biochemical and Biophysical Research Communications %I Elsevier %V 483 %P 1084 - 1095 %8 2017-02-01 %D 2017 %R 10.1016/j.bbrc.2016.09.065 %K Huntington's diseaseNMRS %K pectroscopy %K Mitochondria %K Excitotoxicity %K Calcium %K Glutamate %K NMDA receptors %K Energy metabolism %K Biomarker %Z Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology %Z Life Sciences [q-bio]/Human health and pathology %Z Life Sciences [q-bio]/Bioengineering/ImagingJournal articles %X Huntington's disease (HD) is an inherited progressive neurodegenerative disorder associated with involuntary abnormal movements (chorea), cognitive deficits and psychiatric disturbances. The most striking neuropathological change in HD is the early atrophy of the striatum. While the disease progresses, other brain structures also degenerate, including the cerebral cortex. Changes are also seen outside the brain, in particular weight loss/cachexia despite high dietary intake. The disease is caused by an abnormal expansion of a CAG repeat in the gene encoding the huntingtin protein (Htt). This mutation leads to the expression of a poly-glutamine stretch that changes the biological functions of mutant Htt (mHtt). The mechanisms underlying neurodegeneration in HD are not totally elucidated. Here, we discuss recent results obtained in patients, animal and cellular models suggesting that early disturbance in energy metabolism at least in part associated with mitochondrial defects may play a central role, even though all data are not congruent, possibly because most findings were obtained in cell culture systems or using biochemical analyses of post mortem tissues from rodent models. Thus, we put a particular focus on brain imaging studies that could identify biomarkers of energy defects in vivo and would be of prime interest in preclinical and clinical trials testing the efficacy of new therapies targeting energy metabolism in HD. %G English %L cea-01752431 %U https://cea.hal.science/cea-01752431 %~ CEA %~ CNRS %~ CEA-UPSAY %~ INSERM-SACLAY %~ UNIV-PARIS-SACLAY %~ CEA-UPSAY-SACLAY %~ JACOB %~ CEA-DRF %~ MIRCEN %~ TEST-HALCNRS %~ ANR %~ GS-ENGINEERING