Exp Neurobiol.  2014 Dec;23(4):345-351. 10.5607/en.2014.23.4.345.

Mitochondrial Homeostasis Molecules: Regulation by a Trio of Recessive Parkinson's Disease Genes

• Han JY
• Kim JS
• Son JH

• KMID: 2165539
• DOI: http://doi.org/10.5607/en.2014.23.4.345

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.

Keyword

PD genes; PINK1; Parkin; DJ-1; Mitophagy; Biogenesis

MeSH Terms

Adenosine Triphosphate
Brain
Homeostasis*
Mitochondria
Mitochondrial Degradation
Neurons
Organelles
Parkinson Disease*
Quality Control
Organelle Biogenesis
Adenosine Triphosphate