Gene Symbol: CHCHD3
Description: coiled-coil-helix-coiled-coil-helix domain containing 3
Alias: MINOS3, Mic19, PPP1R22, MICOS complex subunit MIC19, coiled-coil-helix-coiled-coil-helix domain-containing protein 3, mitochondrial, mitochondrial inner membrane organizing system 3, protein phosphatase 1, regulatory subunit 22
Species: human
Products:     CHCHD3

Top Publications

  1. Xie J, Marusich M, Souda P, Whitelegge J, Capaldi R. The mitochondrial inner membrane protein mitofilin exists as a complex with SAM50, metaxins 1 and 2, coiled-coil-helix coiled-coil-helix domain-containing protein 3 and 6 and DnaJC11. FEBS Lett. 2007;581:3545-9 pubmed
    ..This mAb immunocaptures its target protein in association with six other proteins, metaxins 1 and 2, SAM50, CHCHD3, CHCHD6 and DnaJC11, respectively...
  2. Darshi M, Mendiola V, Mackey M, Murphy A, Koller A, Perkins G, et al. ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function. J Biol Chem. 2011;286:2918-32 pubmed publisher
    ..Here, we characterize ChChd3, a previously identified PKA substrate of unknown function (Schauble, S., King, C. C., Darshi, M., Koller, A...
  3. An J, Shi J, He Q, Lui K, Liu Y, Huang Y, et al. CHCM1/CHCHD6, novel mitochondrial protein linked to regulation of mitofilin and mitochondrial cristae morphology. J Biol Chem. 2012;287:7411-26 pubmed publisher
    ..CHCM1/CHCHD6 also interacts with other mitofilin-associated proteins, including DISC1 and CHCHD3. Knockdown of CHCM1/CHCHD6 reduces mitofilin protein levels; conversely, mitofilin knockdown leads to reduction in ..
  4. Ott C, Ross K, Straub S, Thiede B, Götz M, Goosmann C, et al. Sam50 functions in mitochondrial intermembrane space bridging and biogenesis of respiratory complexes. Mol Cell Biol. 2012;32:1173-88 pubmed publisher
    ..evidence that the SAM components exist in a large protein complex together with the IMM proteins mitofilin and CHCHD3, which we term the mitochondrial intermembrane space bridging (MIB) complex...
  5. Xue R, Yu X, Zhao M, Xu M, Wu Q, Cui Y, et al. Pyridostigmine alleviates cardiac dysfunction via improving mitochondrial cristae shape in a mouse model of metabolic syndrome. Free Radic Biol Med. 2019;134:119-132 pubmed publisher
    ..Specifically, PYR upregulated OPA1 and Mfn1/2 expression, promoted the formation of the mitofilin/CHCHD3/Sam50 complex, and decreased p-Drp1 and Fis1 expression, resulting in tight and parallel cristae and increasing ..
  6. Hadsell D, Hadsell L, Olea W, Rijnkels M, Creighton C, Smyth I, et al. In-silico QTL mapping of postpubertal mammary ductal development in the mouse uncovers potential human breast cancer risk loci. Mamm Genome. 2015;26:57-79 pubmed publisher
    ..P = 1 × 10(-11)) association of the study was on MMU6 and contained the genes Plxna4, Plxna4os1, and Chchd3. On MMU5, a QTL was detected (P = 8 × 10(-7)) that was syntenic to a human BrCa locus on h12q24...
  7. Genin E, Plutino M, Bannwarth S, Villa E, Cisneros Barroso E, Roy M, et al. CHCHD10 mutations promote loss of mitochondrial cristae junctions with impaired mitochondrial genome maintenance and inhibition of apoptosis. EMBO Mol Med. 2016;8:58-72 pubmed publisher
    ..Here, we show that CHCHD10 resides with mitofilin, CHCHD3 and CHCHD6 within the "mitochondrial contact site and cristae organizing system" (MICOS) complex...
  8. Darshi M, Trinh K, Murphy A, Taylor S. Targeting and import mechanism of coiled-coil helix coiled-coil helix domain-containing protein 3 (ChChd3) into the mitochondrial intermembrane space. J Biol Chem. 2012;287:39480-91 pubmed publisher
    Coiled-coil helix coiled-coil helix domain-containing protein 3 (ChChd3) is a mitochondrial inner membrane (IM) protein facing toward the intermembrane space (IMS)...
  9. Liu H, Li Y, Li Y, Liu B, Wu H, Wang J, et al. Cloning and functional analysis of FLJ20420: a novel transcription factor for the BAG-1 promoter. PLoS ONE. 2012;7:e34832 pubmed publisher
    ..0001). Taken together, our experiments suggest that FLJ20420 functions as a down-regulator of BAG-1 expression. Its abnormal expression may be involved in the oncogenesis of human malignancies such as lung cancer. ..

More Information


  1. Jans D, Wurm C, Riedel D, Wenzel D, Stagge F, Deckers M, et al. STED super-resolution microscopy reveals an array of MINOS clusters along human mitochondria. Proc Natl Acad Sci U S A. 2013;110:8936-41 pubmed publisher
    ..electron microscopy, we determined the distribution of three known human MINOS subunits (mitofilin, MINOS1, and CHCHD3) in mammalian cells...
  2. Li H, Ruan Y, Zhang K, Jian F, Hu C, Miao L, et al. Mic60/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization. Cell Death Differ. 2016;23:380-92 pubmed publisher
    ..The downregulation of Mic60/Mitofilin or Mic19/CHCHD3 results in instability of other MICOS components, disassembly of MICOS complex and disorganized mitochondrial ..
  3. Sastri M, Darshi M, Mackey M, Ramachandra R, Ju S, Phan S, et al. Sub-mitochondrial localization of the genetic-tagged mitochondrial intermembrane space-bridging components Mic19, Mic60 and Sam50. J Cell Sci. 2017;130:3248-3260 pubmed publisher
    ..tomography, we have mapped at nanoscale resolution three key MIB components, Mic19, Mic60 and Sam50 (also known as CHCHD3, IMMT and SAMM50, respectively), in the environment of structural landmarks such as cristae and crista junctions (..
  4. Guarani V, McNeill E, Paulo J, Huttlin E, Fröhlich F, Gygi S, et al. QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology. elife. 2015;4: pubmed publisher
    ..quantitative proteomics, we show that loss of QIL1 resulted in MICOS disassembly with the accumulation of a MIC60-MIC19-MIC25 sub-complex and degradation of MIC10, MIC26, and MIC27...
  5. Ott C, Dorsch E, Fraunholz M, Straub S, Kozjak Pavlovic V. Detailed analysis of the human mitochondrial contact site complex indicate a hierarchy of subunits. PLoS ONE. 2015;10:e0120213 pubmed publisher
    ..We show that the most important subunits of the MIB complex in human mitochondria are Mic60/Mitofilin, Mic19/CHCHD3 and an outer membrane component Sam50...
  6. Sakowska P, Jans D, Mohanraj K, Riedel D, Jakobs S, Chacinska A. The Oxidation Status of Mic19 Regulates MICOS Assembly. Mol Cell Biol. 2015;35:4222-37 pubmed publisher
    ..However, the mechanisms that underlie MICOS regulation remain poorly understood. MIC19, a peripheral protein of the inner membrane and component of the MICOS complex, was previously reported to be ..
  7. Deng Q, Guo T, Zhou X, Xi Y, Yang X, Ge W. Cross-Talk Between Mitochondrial Fusion and the Hippo Pathway in Controlling Cell Proliferation During Drosophila Development. Genetics. 2016;203:1777-88 pubmed publisher
    ..Here we identify a Drosophila mitochondrial inner membrane protein ChChd3 as a novel regulator for tissue growth. Loss of ChChd3 leads to tissue undergrowth and cell proliferation defects...