Abstract
Mitochondria is essential to generate metabolic energy in eukaryotic cells as well as to regulate calcium buffering, cell signaling, the production of reactive oxygen species (ROS), and apoptosis. They mainly produce most of the cellular energy derived from the breakdown of carbohydrates and fatty acids, which is consequently converted to ATP via oxidative phosphorylation. Mitochondria are also distinctive among the cytoplasmic organelles in that they contain their own DNA, which encodes limited number of mitochondrial proteins, tRNAs, and rRNAs. Evidence has accumulated from many reports, indicating that mitochondrial abnormalities are involved in age-related neurodegenerative diseases (NDDs). Causal factors for most age-related neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), and amyotrophic lateral sclerosis are largely unknown. Although genetic defects are reported to cause a small number of NDDs, cellular, molecular, and pathological mechanisms of disease progression and selective neuronal cell death are not understood fully in these diseases. Especially, age-dependent and mitochondriagenerated ROS has been identified as an important factor that is responsible for disease progression and cell death, particularly in late-onset diseases. Based on the current hypothesis supported by many recent findings, this issue discusses the roles of mitochondria in the progression of age-related neurodegenerative diseases, the connection between mitochondrial abnormalities and NDD, and the drug development targeted to mitochondria in NDDs. [BMB Reports 2020; 53(1): 1-2].