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MICU1 regulates mitochondrial cristae structure and function independently of the mitochondrial Ca<sup>2+</sup> uniporter channel.


ABSTRACT: MICU1 is a calcium (Ca2+)-binding protein that regulates the mitochondrial Ca2+ uniporter channel complex (mtCU) and mitochondrial Ca2+ uptake. MICU1 knockout mice display disorganized mitochondrial architecture, a phenotype that is distinct from that of mice with deficiencies in other mtCU subunits and, thus, is likely not explained by changes in mitochondrial matrix Ca2+ content. Using proteomic and cellular imaging techniques, we found that MICU1 localized to the mitochondrial contact site and cristae organizing system (MICOS) and directly interacted with the MICOS components MIC60 and CHCHD2 independently of the mtCU. We demonstrated that MICU1 was essential for MICOS complex formation and that MICU1 ablation resulted in altered crista

SUBMITTER: Tomar D 

PROVIDER: S-EPMC10388395 | biostudies-literature | 2023 Apr

REPOSITORIES: biostudies-literature