Lon Protease Homolog, Mitochondrial

**Semantic type:** Amino Acid, Peptide, or Protein|Enzyme

**Definition:** Lon protease homolog, mitochondrial (959 aa, ~106 kDa) is encoded by the human LONP1 gene. This protein is involved in the selective degradation of misfolded or damaged polypeptides and of short-lived regulatory proteins.

**Synonyms:** - EC 3.4.21.53 - LONHs - LONP - LONP1 - Lon Peptidase 1, Mitochondrial - Lon Protease-Like Protein - Mitochondrial ATP-Dependent Protease Lon - Mitochondrial Lon Protease-Like Protein - hLON ATP-Dependent Protease