[HTML][HTML] Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats

FN Gellerich, Z Gizatullina, HP Nguyen… - Journal of biological …, 2008 - ASBMB
Huntington disease (HD) is characterized by polyglutamine expansions of huntingtin (htt),
but the underlying pathomechanisms have remained unclear. We studied brain
mitochondria of transgenic HD rats with 51 glutamine repeats (htt 51Q), modeling the adult
form of HD.\(C afree 2+\) up to 2 μm activated state 3 respiration of wild type mitochondria
with glutamate/malate or pyruvate/malate as substrates.\(C afree 2+\) above 2 μm inhibited
respiration via cyclosporin A-dependent permeability transition (PT). Ruthenium red, an …