Lack of age-related clinical progression in PGC-1α-deficient mice - implications for mitochondrial encephalopathies
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- 作者：Levente Szalardya ; levente.szalardy@yahoo.com" class="auth_mail" title="E-mail the corresponding author ; Mate Molnara ; matem87@gmail.com" class="auth_mail" title="E-mail the corresponding author ; Rita Toroka ; torok.rituska@gmail.com" class="auth_mail" title="E-mail the corresponding author ; Denes Zadoria ; zadorid@gmail.com" class="auth_mail" title="E-mail the corresponding author ; Gabor G. Kovacsb ; gabor.kovacs@meduniwien.ac.at" class="auth_mail" title="E-mail the corresponding author ; Laszlo Vecseia ; c ; vecsei.laszlo@med.u-szeged.hu" class="auth_mail" title="E-mail the corresponding author ; Peter Klivenyia ; klivenyi.peter@med.u-szeged.hu" class="auth_mail" title="E-mail the corresponding author
- 关键词：AD ; Alzheimer&rsquo ; s disease ; ALS ; amyotrophic lateral sclerosis ; CNS ; central nervous system ; COX ; cythochrome oxidase ; ERR ; estrogen-related receptor ; FL-PGC-1α ; full-length PGC-1α ; GFAP ; glial fibrillary acidic protein ; HE ; hematoxylin and eosin ; HD ; Huntington&rsquo ; s disease ; KLB ; Klü ; ver-Barrera staining ; KSS ; Kearns-Sayre syndrome ; LS ; Leigh syndrome ; MEF2C ; myocyte-specific enhancer factor 2C ; MELAS ; mitochondrial encephalomyopathy ; lactic acidosis and stroke-like episodes ; MERRF ; m
- 刊名：Behavioural Brain Research
- 出版年：2016
- 出版时间：15 October 2016
- 年：2016
- 卷：313
- 期：Complete
- 页码：272-281
- 全文大小：1770 K
文摘
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FL-PGC-1α -/- mice have a symptomatology corresponding with mitochondrial encephalopathy.
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The behavioral alterations intriguingly do not progress with age.
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The alterations do not associate with retinal or spinal cord involvement.
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The histopathology of skeletal muscle demonstrates only mild myopathic changes.
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CNS-specific isoforms are the predominant PGC-1α mRNAs in the murine brain.