Mitochondrial fission regulator 1 (MTFR1)

Mitochondrial fission regulator 1 (MTFR1) is a poly-proline-rich protein localized to the mitochondria, where it regulates mitochondrial fission and fusion processes integral to cellular energy production, morphology, and stress response[2][3][4][5][13]. The protein is evolutionarily conserved and related to chondrocyte function in certain species. MTFR1 is overexpressed in several cancers, promoting tumor cell proliferation, survival under metabolic stress, and resistance to chemotherapy[2][9]. Its activity is regulated post-translationally, such as via NEK1-mediated phosphorylation, which shifts mitochondrial dynamics toward fusion in response to stress[2]. Given its central role in mitochondrial organization and cellular adaptation, MTFR1 is implicated both as a therapeutic target and a biomarker in oncology and disorders related to mitochondrial dysfunction[2][9][13].

Other names

CHPPRFAM54A2KIAA0009Chondrocyte protein with a poly-proline regionlikely ortholog of chicken chondrocyte protein with a poly proline region

Drugs targeting MTFR1 may modulate mitochondrial fusion/fission balance and affect cell proliferation, survival, and drug resistance in cancer cells