KIAA0391/PRORP Rabbit Polyclonal Antibody for WB and ELISA - O15091

Core Product Specifications and Parameters

Biological Background: KIAA0391/PRORP Function and Localization

• KIAA0391/PRORP, also known as mitochondrial ribonuclease P protein 3 (MRPP3), is the catalytic subunit of mitochondrial RNase P.
• It functions in the RNase P complex with TRMT10C/MRPP1 and HSD17B10/MRPP2 to cleave the 5'-leader sequences from precursor mitochondrial tRNAs. This activity is strictly dependent on the accessory subunits. Related references: PMID:18984158, PMID:25953853, PMID:34715011
• Proper tRNA processing by PRORP is essential for mitochondrial protein synthesis, and mutations in PRORP cause combined oxidative phosphorylation deficiency 54 (COXPD54), a primary mitochondrial disease.
• The protein localizes exclusively to the mitochondrial matrix, consistent with its role in mitochondrial tRNA maturation.
• PRORP is a metal-dependent nuclease requiring Mg²⁺, Mn²⁺, or Zn²⁺ for catalysis, and it contains a mitochondrial transit peptide for import.
• Post-translational modifications include phosphorylation, and the gene undergoes alternative splicing, suggesting regulatory complexity.

Experimental Guidance and Technical Tips

• The immunogen corresponds to the C-terminal region (aa 314-583), which is downstream of the predicted mitochondrial transit peptide, so the antibody likely recognizes the mature form.
• For Western blot, the predicted molecular weight of the full-length protein is ~67 kDa; however, the mature processed form may be smaller. Validate the detected band size using appropriate mitochondrial lysates or recombinant controls.
• Cross-reactivity with rat has been observed, suggesting the antibody may recognize orthologous PRORP in rat samples; verify sequence homology for other species if needed.
• In ELISA, consider using recombinant PRORP protein or mitochondrial extracts as coating antigen; optimize coating concentration and antibody dilution.
• Since PRORP is a mitochondrial protein, efficient protein extraction from mitochondrial fractions may improve detection sensitivity in WB.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio provides diagnostic manufacturers and research labs with reliable access to validated IVD raw materials, including antibodies like this KIAA0391/PRORP polyclonal antibody for studying mitochondrial disorders and tRNA biology. Beyond single antibodies, we offer matched antibody pairs, bulk ancillary reagents, and customized sourcing for rare-target raw materials to support your entire assay development workflow.