Anti-MRPP3 antibody [EPR14321-30]

Supplementary info

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

Activity summary

MRPP3 also known as mitochondrial RNase P protein 3 is part of the RNase P complex which processes precursor tRNA molecules by cleaving their 5' leader sequences. It is a single subunit with a molecular mass of about 58 kDa and is mainly found in mitochondria. The protein shows expression in various tissues indicating its essential role in mitochondrial function.

Biological function summary

MRPP3 plays a critical role in mitochondrial RNA processing acting together with MRPP1 and MRPP2 within the RNase P complex. This complex is responsible for the maturation of mitochondrial tRNAs which are necessary for mitochondrial protein synthesis. MRPP3 is important for the stabilization and function of the RNase P complex ensuring the accuracy of tRNA processing.

Pathways

Mitochondrial RNA processing involves the collaboration of MRPP3 with components such as MRPP1 and MRPP2. This processing is an important part of the mitochondrial translation pathway which governs the synthesis of proteins required for mitochondrial oxidative phosphorylation. MRPP3 intersects with other mitochondrial proteins involved in maintaining proper respiration and energy production.

Associated diseases and disorders

Defects in MRPP3 function are associated with mitochondrial diseases notably those linked to deficiencies in mitochondrial processing. Mutations can impair mitochondrial tRNA processing leading to defects in protein synthesis and energy production implicated in conditions like mitochondrial myopathy. MRPP3's interaction with MRPP1 and MRPP2 is significant as alterations in these proteins can exacerbate mitochondrial disorders.