Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
Reductions in COQ2 Expression Relate to Reduced ATP Levels in Multiple System Atrophy Brain. / Hsiao, Jen Hsiang T.; Purushothuman, Sivaraman; Jensen, Poul H. et al.
In: Frontiers in Neuroscience, Vol. 13, 1187, 01.11.2019.Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Journal article › Research › peer-review
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TY - JOUR
T1 - Reductions in COQ2 Expression Relate to Reduced ATP Levels in Multiple System Atrophy Brain
AU - Hsiao, Jen Hsiang T.
AU - Purushothuman, Sivaraman
AU - Jensen, Poul H.
AU - Halliday, Glenda M.
AU - Kim, Woojin Scott
PY - 2019/11/1
Y1 - 2019/11/1
N2 - Multiple system atrophy (MSA) is a progressive neurodegenerative disease clinically characterized by parkinsonism and cerebellar ataxia, and pathologically by oligodendrocyte α-synuclein inclusions. Genetic variants of COQ2 are associated with an increased risk for MSA in certain populations. Also, deficits in the level of coenzyme Q10 and its biosynthetic enzymes are associated with MSA. Here, we measured ATP levels and expression of biosynthetic enzymes for coenzyme Q10, including COQ2, in multiple regions of MSA and control brains. We found a reduction in ATP levels in disease-affected regions of MSA brain that associated with reduced expression of COQ2 and COQ7, supporting the concept that abnormalities in the biosynthesis of coenzyme Q10 play an important role in the pathogenesis of MSA.
AB - Multiple system atrophy (MSA) is a progressive neurodegenerative disease clinically characterized by parkinsonism and cerebellar ataxia, and pathologically by oligodendrocyte α-synuclein inclusions. Genetic variants of COQ2 are associated with an increased risk for MSA in certain populations. Also, deficits in the level of coenzyme Q10 and its biosynthetic enzymes are associated with MSA. Here, we measured ATP levels and expression of biosynthetic enzymes for coenzyme Q10, including COQ2, in multiple regions of MSA and control brains. We found a reduction in ATP levels in disease-affected regions of MSA brain that associated with reduced expression of COQ2 and COQ7, supporting the concept that abnormalities in the biosynthesis of coenzyme Q10 play an important role in the pathogenesis of MSA.
KW - ATP
KW - cerebellum
KW - coenzyme Q
KW - COQ2
KW - COQ7
KW - multiple system atrophy
KW - parkinsonian disorders
UR - http://www.scopus.com/inward/record.url?scp=85075339409&partnerID=8YFLogxK
U2 - 10.3389/fnins.2019.01187
DO - 10.3389/fnins.2019.01187
M3 - Journal article
C2 - 31736705
AN - SCOPUS:85075339409
VL - 13
JO - Frontiers in Neuroscience
JF - Frontiers in Neuroscience
SN - 1662-4548
M1 - 1187
ER -