Anti-OXA1L antibody

Supplementary info

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

Activity summary

OXA1L also known as mitochondrial inner membrane protein OXA1L functions mechanically in the insertion and assembly of proteins in the mitochondrial inner membrane. This protein with a mass of approximately 45 kDa localizes to the mitochondria and exhibits broad tissue expression. OXA1L plays a role in protein translocation specifically contributing to the insertion of multi-spanning membrane proteins coming from the mitochondrial ribosomes.

Biological function summary

OXA1L facilitates the integration of proteins into the mitochondrial inner membrane essential for maintaining mitochondrial function and biogenesis. OXA1L operates as part of a larger complex that assists protein assembly and stabilization. Such interactions highlight its role in mitochondrial respiratory chain assembly necessary for maintaining cellular energy balance.

Pathways

OXA1L integrates specifically into the pathway of mitochondrial oxidative phosphorylation. This target works alongside important proteins like COX1 and COX2 shaping the proper assembly of components of the electron transport chain. Additionally OXA1L interacts with the mitochondrial import machinery vital for maintaining the efficiency of protein import pathways that regulate cellular respiration.

Associated diseases and disorders

Mutations or dysregulation of OXA1L correlate with mitochondrial disorders and neurological diseases including sensorineural hearing loss and mitochondrial complex I deficiency. NDUFAF2 involved in complex I assembly shows interaction with OXA1L in these conditions highlighting a shared pathway that can lead to disrupted energy production and neurological symptoms.