, 2011, no
A range of disorders have been recognized: varying from psychomotor retardation in childhood, to intermittent neuropsychiatric disease with ataxia and other motor disabilities, such as Friedreich's and other spinocerebellar ataxias [70], as well as neural diseases where mental deficits are also visible such as Parkinson's disease, and Alzheimer's disease (AD) [70]In Parkinsons Disease which has been deeply investigated, KGDHC Activity is reduced, coupled to elevated levels of monoamine oxidase B [71] and cytosolic accumulation of cytochrome c which inturn activates other pathways, including cell death cascades and enzyme inhibition which alters Ca2+homeostasis [72] The KGDHC enzyme is further a target for ubiquitination-dependent degradation in mitochondria by binding of Siah2, the RING finger ubiquitin-protein isopeptide ligase 2, encoded by gene siah2 [73]
This increases oxidative stress, and leaves the body vulnerable to further damage from toxins
[DOI] [PubMed] [Google Scholar] Zhou JH, Wang XT, Zhou Liang, Zhou Lin, Xu FX, Su LD, Wang H, Jia F, Xu FQ, Chen GQ, De Zeeuw CI, Shen Y
