Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model.

Lodewijk J A ToonenMaurice OverzierMelvin M EversLeticia G LeonSander A J van der ZeeuwHailiang MeiSzymon M KielbasaJelle J GoemanKristina M HettneOlafur Th MagnussonMarion PoirelAlexandre SeyerPeter A C 't HoenWilleke M C van Roon-Mom

Published in: Molecular neurodegeneration (2018)

The observed transcriptional changes in SCA3 mouse brain reveal parallels with previous reported neuropathology in patients, but also shows brain region specific effects as well as involvement of adrenergic signalling and CREB pathway changes in SCA3. Importantly, the transcriptional changes occur prior to onset of motor- and coordination deficits.

Keyphrases

mouse model
gene expression
end stage renal disease
transcription factor
single cell
newly diagnosed
ejection fraction
chronic kidney disease
traumatic brain injury
heat shock
peritoneal dialysis
prognostic factors
early onset
resting state
multiple sclerosis
oxidative stress
dna methylation
functional connectivity