Recombinant Human TIMM23/TIM23 protein (GST tag N-Terminus)

補足情報

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TIMM23 also known as TIM23 protein is a critical component of the mitochondrial import machinery. It weighs approximately 24 kDa and is expressed in the inner mitochondrial membrane. TIMM23 is part of the translocase of inner mitochondrial membrane (TIM) complex which is essential for translocating proteins from the mitochondrial intermembrane space into the matrix. The TIM23 complex facilitates the passage of pre-sequence or matrix-targeted proteins that have signal sequences recognizable by the mitochondrial import machinery.

Biological function summary

TIMM23 impacts mitochondrial function by cooperating with the TIM complex to ensure the proper import and processing of essential mitochondrial proteins. The complex known to include other subunits like TIMM17 and TIMM50 organizes the translocase activity required for maintaining mitochondrial homeostasis. TIMM23’s function is pivotal for energy production as it enables the importation of proteins important to several enzymatic processes particularly in oxidative phosphorylation.

Pathways

Proteins like TIMM23 play a role in maintaining mitochondrial integrity which is essential for metabolic pathways. In particular TIMM23 is integral to the oxidative phosphorylation pathway linking it with proteins such as ATP synthase involved in ATP production. The electron transport chain also depends indirectly on TIMM23 for ensuring that necessary proteins reach the inner mitochondrial membrane implicating TIMM23 in cellular respiration processes.

TIMM23 aberrations can result in mitochondrial dysfunction contributing to conditions such as mitochondrial myopathy. The disruption in protein import can affect energy metabolism which is a common feature in diseases like Leigh syndrome. Other proteins such as complex I and complex IV of the electron transport chain can also exhibit dysfunction when affected by a compromised TIMM23 function highlighting its role in maintaining cellular energy efficiency.