Depletion of mitochondrial DNA in leukocytes of patients with poly-Q diseases

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Title:	Depletion of mitochondrial DNA in leukocytes of patients with poly-Q diseases
Authors:	劉青山(Liu Chin-San)、鄭文玲(CHENG Wen-Ling)、郭守仁(KUO Shou-Jen)、李介元(Jie-Yuan Li)、宋秉文(Soong Bing-Wen)、魏耀揮(Wei Yau-Huei)*
Contributors:	中醫學院中西醫結合研究所
Keywords:	Copy number;MitochondrialDNA;Polyglutamine;CAG repeat numbers;Leukocyte;Neurodegenerative disorders
Date:	2008-01-15
Issue Date:	2009-08-19 16:34:09 (UTC+8)
Abstract:	Polyglutamine (poly-Q) diseases are late-onset neurodegenerative disorders arising from the expansion of an unstable CAG repeat in the affected gene, which is translated to a tract of glutamine residues. This kind of mutant proteins may be aggregated and accumulated, and thereby enhance cellular oxidative stress. In one of our previous studies (Free Radic. Res. 2003;37:1307-17), we found that alteration in the leukocyte mtDNA content is very sensitive to the level of oxidative stress in blood. Thus, we proposed that leukocyte mtDNA content may be used as a biomarker to predict the severity of clinical manifestation of poly-Qdiseases. We recruited 50 healthy subjects and 114 patients with poly-Qdiseases, including spinal cerebellar atrophy 2/3, spinal bulbar muscular atrophy, and Huntington chorea. We found that mtDNA in leukocytes was depleted in patients with poly-Qdiseases (P < 0.05). Moreover, the results showed that patients with lower mtDNA content more frequently manifested multiple-symptom disorders and had high CAG repeat numbers in the mutant genes. In conclusion, we suggest that leukocyte mtDNA content correlates with the length of GAG repeat and may serve as an index of the severity of poly-Qdiseases.
Relation:	JOURNAL OF THE NEUROLOGICAL SCIENCES 264(1-2):18~21
Appears in Collections:	[Graduate Institute of Integrated Medicine] Journal articles

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