LRPPRC抗体—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human colon cancer tissue using P11007(LRPPRC Antibody) at dilution 1/15. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using P11007(LRPPRC Antibody) at dilution 1/15. (Original magnification: ×200)    

  
  

The LRPPRC (Leucine-Rich Pentatricopeptide Repeat Containing) protein is a critical mitochondrial protein involved in RNA processing, stabilization, and translation. It plays a key role in maintaining mitochondrial mRNA stability and coordinating the assembly of mitochondrial ribosomes. Mutations in the LRPPRC gene are linked to Leigh syndrome French Canadian type (LSFC), a severe neurological disorder characterized by impaired mitochondrial energy production.

LRPPRC antibodies are essential tools for studying mitochondrial gene regulation and disease mechanisms. These antibodies enable detection and localization of LRPPRC in tissues or cultured cells via techniques like Western blotting, immunohistochemistry, and immunofluorescence. Researchers use them to investigate LRPPRC's interaction with SLIRP (a stabilizing partner) and its role in post-transcriptional regulation of mitochondrial transcripts.

Studies utilizing LRPPRC antibodies have revealed its tissue-specific expression patterns and reduced levels in LSFC patients, correlating with respiratory chain deficiencies. Such findings underscore its importance in mitochondrial metabolism and pathophysiology. Additionally, these antibodies aid in exploring LRPPRC's emerging roles beyond mitochondria, including potential involvement in cancer progression and cellular stress responses. The development of high-specificity LRPPRC antibodies continues to advance research into mitochondrial disorders and therapeutic strategies.