Mitoregulin (MTLN)

Mitoregulin (MTLN) is a **small micropeptide encoded by the LINC00116 locus**, previously annotated as a long non-coding RNA. It localizes mainly to the mitochondrial outer membrane, with a transmembrane N-terminal region facing the intermembrane space and a C-terminus exposed for interactions. Mitoregulin's established molecular roles center on **maintenance of mitochondrial integrity, lipid homeostasis, and regulation of respiratory complex activities**, particularly involving cardiolipin content and complex I-dependent mitochondrial respiration[1][2]. It interacts with a range of mitochondrial proteins — including those involved in fatty acid metabolism (e.g., HADHA/B), lipid transfer (e.g., CYB5R3, CPT1B, ACSL1), and structure (e.g., MTCH2, MARCH5) — as well as with membrane lipids such as cardiolipin[1]. Knockout and knockdown studies in mice link mitoregulin deficiency to **impaired lipid and energy metabolism, increased oxidative stress, disrupted mitochondrial structural organization (notably creatine kinase oligomerization and membrane tethering), and heightened susceptibility to obesity and kidney fibrosis** under certain stress conditions[1][2]. The detailed pathophysiologic and therapeutic targeting potential of mitoregulin in humans remains under active investigation, and—according to current knowledge—there are no approved drugs or selective small-molecule modulators that interact with MTLN, nor established diagnostic or predictive biomarkers[1][2].

Other names

LEMPLINC00116MPMNCRNA00116MOXITILRMicropeptide in mitochondriaMicropeptide regulator of beta-oxidationSmall integral membrane protein 37lncRNA-encoded micropeptidemicropeptide regulator of β-oxidationTP53-inhibiting lncRNASMIM37long intergenic non-protein coding RNA 116