Mitochondrial Homeostasis Molecules: Regulation by a Trio of Recessive Parkinson's Disease Genes.
- Author:
Ji Young HAN
1
;
Ji Soo KIM
;
Jin H SON
Author Information
- Publication Type:Review
- Keywords: PD genes; PINK1; Parkin; DJ-1; Mitophagy; Biogenesis
- MeSH: Adenosine Triphosphate; Brain; Homeostasis*; Mitochondria; Mitochondrial Degradation; Neurons; Organelles; Parkinson Disease*; Quality Control; Organelle Biogenesis
- From:Experimental Neurobiology 2014;23(4):345-351
- CountryRepublic of Korea
- Language:English
- Abstract: Mitochondria are small organelles that produce the majority of cellular energy as ATP. Mitochondrial dysfunction has been implicated in the pathogenesis of Parkinson's disease (PD), and rare familial forms of PD provide valuable insight into the pathogenic mechanism underlying mitochondrial impairment, even though the majority of PD cases are sporadic. The regulation of mitochondria is crucial for the maintenance of energy-demanding neuronal functions in the brain. Mitochondrial biogenesis and mitophagic degradation are the major regulatory pathways that preserve optimal mitochondrial content, structure and function. In this mini-review, we provide an overview of the mitochondrial quality control mechanisms, emphasizing regulatory molecules in mitophagy and biogenesis that specifically interact with the protein products of three major recessive familial PD genes, PINK1, Parkin and DJ-1.
