MTX1 (metaxin 1) encodes a mitochondrial outer membrane protein essential for mitochondrial protein import and cellular function 1. The protein contains 317 amino acids, lacks an N-terminal signal sequence, but possesses a putative transmembrane domain near its C-terminus, consistent with its mitochondrial localization 1. MTX1 is involved in protein transport into mitochondria and is essential for embryonic development 1. The gene exhibits a unique genomic organization, sharing a common promoter region with the thrombospondin 3 (THBS3) gene and being transcribed convergently 21. Functionally, MTX1 promotes autophagy through interaction with CDGSH iron sulfur domain 1 (CISD1), an autophagy negative regulator 3. Clinically, MTX1 overexpression is associated with hepatocellular carcinoma progression and sorafenib resistance, with high expression correlating with poor outcomes in HCC patients receiving sorafenib treatment 3. Additionally, specific MTX1 variants may modify disease onset in GBA-associated Parkinson's disease, with the homozygous c.184A/A genotype associated with earlier symptom onset 4. Recent studies also implicate MTX1 in idiopathic pulmonary fibrosis pathogenesis 5.