Mitochondrial biology and the identification of biomarkers of Huntington's disease

被引：6

作者：

Neueder, Andreas ^{[1
]}

Orth, Michael ^{[1
,2
]}

机构：

[1] Ulm Univ, Dept Neurol, Ulm, Germany

[2] Neurozentrum Siloah, SwissHuntingtons Dis Ctr, Worbstr 312, CH-3073 Gumligenbei Bern, Switzerland

来源：

NEURODEGENERATIVE DISEASE MANAGEMENT | 2020年 / 10卷 / 04期

关键词：

fission and fusion; huntingtin fragment; mitochondrial quality control; mtDNA; splicing; TRANSCRIPTION FACTOR-A; ISOLATED BRAIN MITOCHONDRIA; CYTOCHROME-C RELEASE; YAC128 MOUSE MODEL; COMPLEX-II DEFECTS; DNA COPY NUMBER; MUTANT HUNTINGTIN; STRIATAL NEURONS; CAG REPEAT; NUCLEAR-LOCALIZATION;

D O I：

10.2217/nmt-2019-0033

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Apart from finding novel compounds for treating Huntington's disease (HD) an important challenge at present consists in finding reliable read-outs or biomarkers that reflect key biological processes involved in HD pathogenesis. The core elements of HD biology, for example, HTT RNA levels or protein species can serve as biomarker, as could measures from biological systems or pathways in which Huntingtin plays an important role. Here we review the evidence for the involvement of mitochondrial biology in HD. The most consistent findings pertain to mitochondrial quality control, for example, fission/fusion. However, a convincing mitochondrial signature with biomarker potential is yet to emerge. This requires more research including in peripheral sources of human material, such as blood, or skeletal muscle.

引用

页码：243 / 255

页数：13

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