| P50 | author | Raymund A. C. Roos | Q67223099 |
| | Chiara Milanese | Q80805100 |
| | Ronald Buijsen | Q83275003 |
| P2093 | author name string | Willeke M C van Roon-Mom | |
| | N Ahmad Aziz | |
| | Sarah L Gardiner | |
| | Pier G Mastroberardino | |
| | Merel W Boogaard | |
| | Marye Hogenboom | |
| P2860 | cites work | Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III. | Q45302461 |
| | Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. | Q45302702 |
| | Acute glutathione depletion restricts mitochondrial ATP export in cerebellar granule neurons. | Q45973938 |
| | Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid. | Q48365871 |
| | Bioenergetic and proteolytic defects in fibroblasts from patients with sporadic Parkinson's disease. | Q48773342 |
| | Induction, Effects, and Quantification of Sublethal Oxidative Stress by Hydrogen Peroxide on Cultured Human Fibroblasts | Q63473080 |
| | Delayed dystonia with striatal CT lucencies induced by a mycotoxin (3-nitropropionic acid) | Q71631143 |
| | Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization | Q95810356 |
| | A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 |
| | Early energy deficit in Huntington disease: identification of a plasma biomarker traceable during disease progression | Q33291754 |
| | Replicating Huntington's disease phenotype in experimental animals | Q33749061 |
| | Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation | Q34042260 |
| | Age-at-onset in Huntington disease | Q34210139 |
| | Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease | Q34373713 |
| | Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin | Q34483577 |
| | Localization of sequence variations in PGC-1α influence their modifying effect in Huntington disease. | Q34498391 |
| | CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches | Q34624785 |
| | The role of mitochondria in aging | Q36638140 |
| | 3-Nitropropionic acid-exogenous animal neurotoxin and possible human striatal toxin. | Q36934346 |
| | Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset | Q37073913 |
| | Mutant huntingtin and mitochondrial dysfunction | Q37308357 |
| | Mitochondrial DNA damage is associated with reduced mitochondrial bioenergetics in Huntington's disease. | Q37355444 |
| | Mitochondrial dysfunction, metabolic deficits, and increased oxidative stress in Huntington's disease. | Q37871649 |
| | Genetic modifiers of Huntington's disease | Q38243002 |
| | Body weight is a robust predictor of clinical progression in Huntington disease | Q38639328 |
| | Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release | Q40551652 |
| | Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. | Q40721588 |
| | Regional mitochondrial respiratory activity in Huntington's disease brain | Q41360902 |
| | Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. | Q42438521 |
| | Spare respiratory capacity rather than oxidative stress regulates glutamate excitotoxicity after partial respiratory inhibition of mitochondrial complex I with rotenone. | Q42515219 |
| | PGC-1alpha as modifier of onset age in Huntington disease | Q42927080 |
| | Metabolic network abnormalities in early Huntington's disease: an [(18)F]FDG PET study. | Q43790732 |
| | Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease | Q43830045 |
| | Oxidative alpha-ketoglutarate dehydrogenase inhibition via subtle elevations in monoamine oxidase B levels results in loss of spare respiratory capacity: implications for Parkinson's disease | Q44578335 |
| | Weight loss in Huntington disease increases with higher CAG repeat number | Q45291411 |
| | Mitochondrial defect in Huntington's disease caudate nucleus | Q45291734 |
| | Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. | Q45293805 |
| | Biochemical abnormalities and excitotoxicity in Huntington's disease brain. | Q45297145 |
| | Oxidative stress in Huntington's disease | Q45297267 |
| | HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism | Q45298206 |
| | Brain white-matter volume loss and glucose hypometabolism precede the clinical symptoms of Huntington's disease. | Q45299986 |
| P433 | issue | 5 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | Huntington's disease | Q190564 |
| P304 | page(s) | e275 | |
| P577 | publication date | 2018-10-04 | |
| P1433 | published in | Neurology. Genetics | Q27727179 |
| P1476 | title | Bioenergetics in fibroblasts of patients with Huntington disease are associated with age at onset | |
| P478 | volume | 4 | |