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FLASH GENE
Symbol PPARGC1A contributors: mct/shn - updated : 21-06-2017
HGNC name peroxisome proliferator-activated receptor gamma, coactivator 1 alpha
HGNC id 9237
ANIMAL & CELL MODELS
  • PGC-1alpha null mice are lean and resistant to diet-induced obesity, constitutively activate gluconeogenic gene expression, and have elevated C/EBPbeta in liver
  • transverse aortic constriction in mice lacking PGC-1alpha leads to accelerated cardiac dysfunction accompanied by signs of significant clinical heart failure
  • mice lacking PGC-1alpha show abnormal diurnal rhythms of activity, body temperature and metabolic rate
  • mice with combined deficiency of PGC-1alpha and PGC-1beta died shortly after birth with small hearts, bradycardia, intermittent heart block, and a markedly reduced cardiac output
  • impaired function of PGC-1alpha plays a critical role in muscle dysfunction in Huntington's disease transgenic mice
  • expression levels of PGC-1 alpha is significantly decreased in both Alzheimer's disease hippocampal tissues and AD-causing amyloid precursor protein mutant M17 cells
  • knockdown of PGC-1 alpha could exacerbate impaired mitochondrial biogenesis and mitochondrial deficits in AD-causing amyloid precursor protein mutant M17 cells
  • mice that lack cardiac PGC-1 alpha, a powerful regulator of angiogenesis, develop profound peripartum cardiomyopathy