Reduced gluconeogenesis and lactate clearance in Huntington's disease

Knud Elnegaard Josefsen, Signe M B Nielsen, André Campos, Thomas Seifert, Lis Frydenreich Hasholt, Jørgen E Nielsen, Anne Nørremølle, Niels Henning Skotte, Niels H Secher, Bjørn Quistorff

36 Citations (Scopus)


We studied systemic and brain glucose and lactate metabolism in Huntington's disease (HD) patients in response to ergometer cycling. Following termination of exercise, blood glucose increased abruptly in control subjects, but no peak was seen in any of the HD patients (2.0 ± 0.5 vs. 0.0 ± 0.2mM, P <2 × 10(-6)). No difference was seen in brain metabolism parameters. Reduced hepatic glucose output in the HD mouse model R6/2 following a lactate challenge, combined with reduced phosphoenolpyruvate carboxykinase and increased pyruvate kinase activity in the mouse liver suggest a reduced capacity for gluconeogenesis in HD, possibly contributing to the clinical symptoms of HD. We propose that blood glucose concentration in the recovery from exercise can be applied as a liver function test in HD patients.
Original languageEnglish
JournalNeurobiology of Disease
Issue number3
Pages (from-to)656-62
Number of pages7
Publication statusPublished - 1 Dec 2010


Dive into the research topics of 'Reduced gluconeogenesis and lactate clearance in Huntington's disease'. Together they form a unique fingerprint.

Cite this