MIPEP Antibody (166CT22.3.7): sc-517345

MIPEP Antibody (166CT22.3.7) is a mouse monoclonal IgG1 kappa light chain antibody that detects the MIPEP protein of human origin by western blotting (WB) and immunoprecipitation (IP). Anti-MIPEP antibody (166CT22.3.7) is available as a non-conjugated format. MIPEP, or mitochondrial intermediate peptidase, is a crucial 713 amino acid enzyme that plays a vital role in the final step of processing nuclear-encoded proteins destined for the mitochondrial inner membrane or matrix. MIPEP (166CT22.3.7) monoclonal antibody enables research into this essential protein for the maturation of oxidative phosphorylation-related proteins, which are critical for cellular energy production, and is predominantly expressed in tissues with high oxygen consumption, such as skeletal muscle, brain, heart, and pancreas. MIPEP′s importance extends beyond its enzymatic function; MIPEP is also the functional homolog of yeast YMIP, a protein that facilitates mitochondrial iron uptake and is regulated by the yeast homolog of frataxin, which is necessary for mitochondrial iron efflux. Given the regulatory influence that human frataxin has on MIPEP, MIPEP may be implicated in the pathophysiology of Friedreich ataxia, an autosomal recessive neurodegenerative disorder linked to frataxin deficiency. MIPEP (166CT22.3.7) antibody thus serves as a valuable tool for researchers investigating mitochondrial function and related diseases.

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MIPEP Antibody (166CT22.3.7) References:

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4. Sequence analysis of rat mitochondrial intermediate peptidase: similarity to zinc metallopeptidases and to a putative yeast homologue.  |  Isaya, G., et al. 1992. Proc Natl Acad Sci U S A. 89: 8317-21. PMID: 1518864
5. Mutation detection in four candidate genes (OXA1L, MRS2L, YME1L and MIPEP) for combined deficiencies in the oxidative phosphorylation system.  |  Coenen, MJ., et al. 2005. J Inherit Metab Dis. 28: 1091-7. PMID: 16435202
6. ncRNA-Encoded Peptides or Proteins and Cancer.  |  Wang, J., et al. 2019. Mol Ther. 27: 1718-1725. PMID: 31526596
7. Variants in the MIPEP gene presenting with complex neurological phenotype without cardiomyopathy, impair OXPHOS protein maturation and lead to a reduced OXPHOS abundance in patient cells.  |  Pulman, J., et al. 2021. Mol Genet Metab. 134: 267-273. PMID: 34620555
8. Case report: Rare novel MIPEP compound heterozygous variants presenting with hypertrophic cardiomyopathy, severe lactic acidosis and hypotonia in a Chinese infant.  |  Wang, L., et al. 2022. Front Cardiovasc Med. 9: 1095882. PMID: 36727025
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