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AFG1-like ATPase (AFG1L) is a mitochondrial AAA+ ATPase and integral membrane protein involved in protein quality control within the mitochondrial matrix and at the inner mitochondrial membrane[3][4][5][1]. It is crucial for the degradation of misfolded or excess nuclear-encoded respiratory complex IV (cytochrome c oxidase) subunits and for maintaining mitochondrial morphology and proteostasis[3][5][4]. Its activity safeguards mitochondrial fidelity, supports oxidative stress tolerance, and preserves overall cellular and organismal health, as shown in yeast and metazoan models[1][5]. In humans, the protein is referred to as LACE1, and variants in the gene have been implicated in disorders such as bipolar disorder and marasmus[3]. There are no known direct pharmacological modulators or drugs that target AFG1-like ATPase.
Not applicable. No drugs directly target this protein as of the current literature. Its mechanisms involve ATP hydrolysis–dependent unfolding and degradation of misfolded mitochondrial proteins, and aiding turnover/extraction of inner mitochondrial membrane protein subunits[1][5].
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