|Other Names||MICOS complex subunit MIC19, Coiled-coil-helix-coiled-coil-helix domain-containing protein 3, CHCHD3, MIC19, MINOS3|
|Target/Specificity||The synthetic peptide sequence is selected from aa 83-96 of HUMAN CHCHD3|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription.|
|Cellular Location||Mitochondrion inner membrane; Lipid-anchor; Intermembrane side Cytoplasm. Nucleus|
|Tissue Location||Detected at low levels in brain, placenta, lung, liver, kidney and pancreas with increased levels in heart and skeletal muscle. Higher expression in primary lung cancers than in normal lung tissue.|
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Required for maintenance of mitochondrial crista integrity and mitochondrial function. May act as a scaffolding protein that stabilizes protein complexes involved in crista architecture and protein import. Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription.
Ota T.,et al.Nat. Genet. 36:40-45(2004).
Daub H.,et al.Mol. Cell 31:438-448(2008).
Dephoure N.,et al.Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008).
Mayya V.,et al.Sci. Signal. 2:RA46-RA46(2009).
Choudhary C.,et al.Science 325:834-840(2009).
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