Anti-TIMM8A/DDP antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

TIMM8A also known as DDP is a protein involved in mitochondrial import machinery important for transporting specific precursor proteins across the intermembrane space to the inner membrane. It has a molecular mass of about 10 kilodaltons. TIMM8A is predominantly expressed in mitochondria facilitating protein translocation through its interaction with carrier proteins. The protein is part of the TIM8-TIM13 complex important for delivering substrates to the TIM23 complex in the inner mitochondrial membrane ensuring the proper functioning of mitochondrial processes.

Biological function summary

In the context of mitochondria-dependent functions TIMM8A acts as a chaperone. It is a component of the TIM22 translocase complex that plays a critical role in inserting metabolite carrier proteins into the inner mitochondrial membrane. This activity is essential for maintaining mitochondrial integrity and ensuring energy production processes occur efficiently. TIMM8A partners with TIMM13 to form a hetero-oligomeric complex highlighting its integral role in translocating necessary proteins.

Pathways

Especially those related to cellular respiration and energy metabolism TIMM8A integrates into the mitochondrial import pathway. Its function relates closely to the TIM22 pathway which is responsible for the translocation and assembly of inner membrane carriers. TIMM8A cooperates with proteins such as TIMM9 and TIMM13 forming a complex that stabilizes the import intermediates. This cooperation ensures that imported proteins navigate correctly to their functional sites within mitochondria.

Associated diseases and disorders

Proteins similar to TIMM8A are associated with Mohr-Tranebjaerg syndrome a disorder characterized by auditory and motor disturbances. Mutations affecting TIMM8A lead to dysfunction in the import machinery contributing to the pathophysiology of the syndrome. Furthermore its association with deafness-dystonia syndrome highlights TIMM8A's involvement in neurodegenerative conditions. These connections suggest potential interactions with related proteins like OPA1 known to impact mitochondrial dynamics and integrity further implicating TIMM8A in maintaining neuronal health.