CHCHD3 (GeneName), MICOS complex subunit MIC19 (ProteinName), MIC19_HUMAN.
Human CHCHD3/ MICOS complex subunit MIC19 ELISA Kit
Coiled-coil-helix-coiled-coil-helix domain-containing protein 3, MIC19, MINOS3
Natural and recombinant human MICOS complex subunit MIC19
Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids
Component of the mitochondrial contact site and cristae organizing system (MICOS) complex, composed of at least MINOS1/MIC10, CHCHD3/MIC19, CHCHD6/MIC25, APOOL/MIC27, IMMT/MIC60, APOO/MIC23/MIC26 and QIL1/MIC13 (PubMed:21081504, PubMed:22114354, PubMed:22228767, PubMed:25997101). This complex was also known under the names MINOS or MitOS complex. The MICOS complex associates with mitochondrial outer membrane proteins SAMM50, MTX1, MTX2 and DNAJC11, mitochondrial inner membrane protein TMEM11 and with HSPA9 (PubMed:21081504, PubMed:22114354, PubMed:22228767, PubMed:25997101). Found in a complex with IMMT/MIC60 and SAMM50 termed mitochondrial intermembrane space bridging (MIB) complex. Interacts with HSPA1A/HSPA1B and OPA1, preferentially with the soluble OPA1 form (By similarity). Interacts with IMMT/MIC60.
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. Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology (PubMed:25781180).
Mitochondrion inner membrane Lipid-anchor Intermembrane side Cytoplasm Nucleus Mitochondrion
MGGTTSTRRV | TFEADENENI | TVVKGIRLSE | NVIDRMKESS | PSGSKSQRYS |
GAYGASVSDE | ELKRRVAEEL | ALEQAKKESE | DQKRLKQAKE | LDRERAAANE |
QLTRAILRER | ICSEEERAKA | KHLARQLEEK | DRVLKKQDAF | YKEQLARLEE |
RSSEFYRVTT | EQYQKAAEEV | EAKFKRYESH | PVCADLQAKI | LQCYRENTHQ |
TLKCSALATQ | YMHCVNHAKQ | SMLEKGG
This product has not yet been referenced specifically in any publications.
"Sam50 functions in mitochondrial intermembrane space bridging and biogenesis of respiratory complexes."
"ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function."
"Detailed analysis of the human mitochondrial contact site complex indicate a hierarchy of subunits."
"QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology."
"Targeting and import mechanism of coiled-coil helix coiled-coil helix domain-containing protein 3 (ChChd3) into the mitochondrial intermembrane space."
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