Anti-LRP130 Antibody

Rabbit polyclonal reagent from Antibodies.com targeting LRP130, a mitochondrial leucine-rich pentatricopeptide repeat protein involved in mitochondrial mRNA stability and linked to the French-Canadian form of Leigh syndrome. Supplied as 100 µl of unconjugated antibody raised in rabbit, validated for western blot, immunohistochemistry, and immunofluorescence workflows on human samples. The protein functions as a post-transcriptional regulator of mitochondrial-encoded transcripts, and loss-of-function mutations in its gene are associated with cytochrome c oxidase deficiency. Useful for investigating mitochondrial gene expression, RNA processing, and the molecular pathology of inherited mitochondrial disorders in research use only settings.