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KMID : 0892920130220040283
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Experimental Neurobiology
2013 Volume.22 No. 4 p.283 ~ p.300
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Mitochondrial Dysfunction of Immortalized Human Adipose Tissue-Derived Mesenchymal Stromal Cells from Patients with Parkinson's Disease
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Moon Hyo-Eun
Yoon Seung-Hee
Hur Yong-Suk
Park Hyung-Woo
Ha Ji-Young
Kim Kyung-Hee
Shim Jung-Hee
Yoo Seung-Hyun
Son Jin-H.
Paek Seung-Leal
Kim In-Keyoung
Hwang Jae-Ha
Kim Dong-Gyu
Kim Han-Joon
Jeon Beom-Seok
Park Sung-Sup
Paek Sun-Ha
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Abstract
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Mitochondrial dysfunction in dopaminergic neurons of patients with idiopathic and familial Parkinson's disease (PD) is well known although the underlying mechanism is not clear. We established a homogeneous population of human adipose tissue-derived mesenchymal stromal cells (hAD-MSCs) from human adult patients with early-onset hereditary familial Parkin-defect PD as well as late-onset idiopathic PD by immortalizing cells with the hTERT gene to better understand the underlying mechanism of PD. The hAD-MSCs from patients with idiopathic PD were designated as "PD", from patients with Parkin-defect PD as "Parkin" and from patients with pituitary adenomas as "non-PD" in short. The pGRN145 plasmid containing hTERT was introduced to establish telomerase immortalized cells. The established hTERT-immortalized cell lines showed chromosomal aneuploidy sustained stably over two-years. The morphological study of mitochondria in the primary and immortalized hAD-MSCs showed that the mitochondria of the non-PD were normal; however, those of the PD and Parkin were gradually damaged. A striking decrease in mitochondrial complex I, II, and IV activities was observed in the hTERT-immortalized cells from the patients with idiopathic and Parkin-defect PD. Comparative Western blot analyses were performed to investigate the expressions of PD specific marker proteins in the hTERT-immortalized cell lines. This study suggests that the hTERT-immortalized hAD-MSC cell lines established from patients with idiopathic and familial Parkin-defect PD could be good cellular models to evaluate mitochondrial dysfunction to better understand the pathogenesis of PD and to develop early diagnostic markers and effective therapy targets for the treatment of PD.
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KEYWORD
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hTERT, hAD-MSC, immortalization, Parkinson's disease, diagnosis
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