LONP3

LONP3, also known as Longevity gene 3, is a protein-coding gene found in humans and other mammals. The protein encoded by LONP3 is a type of AAA+ ATPase, a family of proteins involved in various cellular processes, including DNA replication, protein folding, and protein degradation. Specifically, LONP3 is a mitochondrial matrix protein, meaning it functions within the inner compartment of the cell's mitochondria.

Research suggests that LONP3 plays a crucial role in maintaining mitochondrial health and function. It has been implicated in the removal of damaged or misfolded proteins from the mitochondria, a process essential for preventing cellular dysfunction and the accumulation of toxic aggregates. This protein degradation activity is thought to contribute to cellular longevity and resistance to stress.

Mutations in the LONP3 gene have been associated with certain neurodegenerative diseases, although the exact mechanisms are still under investigation. Studies have explored its involvement in conditions like amyotrophic lateral sclerosis (ALS) and Parkinson's disease, where mitochondrial dysfunction is a common feature. Further research is ongoing to fully elucidate the precise functions of LONP3 and its implications in human health and disease.