TIMM23

TIMM23 refers to a protein translocase subunit found in the inner mitochondrial membrane (IMM). It is a key component of the TIM23 complex, which is responsible for the translocation of nuclear-encoded preproteins into the mitochondrial matrix and insertion of some proteins into the IMM.

The TIM23 complex is a multi-subunit protein complex composed of both core components, such as Tim23 itself, and associated components. It plays a vital role in mitochondrial biogenesis and function by facilitating the import and proper localization of proteins essential for oxidative phosphorylation, metabolism, and other critical processes within the mitochondria.

The translocation mechanism mediated by the TIM23 complex involves the preprotein's signal sequence interacting with the Tim23 channel. This interaction initiates the passage of the preprotein across the IMM. Chaperone proteins within the matrix, such as mtHsp70, assist in pulling the preprotein through the channel and promoting its folding into its mature conformation.

Dysfunction or mutations in TIMM23 can lead to impaired mitochondrial protein import, resulting in various mitochondrial disorders. These disorders can manifest as a wide range of clinical symptoms, affecting different organ systems, depending on the severity and specific impact on mitochondrial function. Research on TIMM23 is ongoing to better understand its role in mitochondrial protein import and to develop potential therapeutic strategies for mitochondrial diseases associated with its dysfunction.