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Protein

MICOS complex subunit MIC25

Gene

CHCHD6

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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.1 Publication

GO - Biological processi

  • cellular response to DNA damage stimulus Source: UniProtKB
  • cristae formation Source: UniProtKB
Complete GO annotation...

Names & Taxonomyi

Protein namesi
Recommended name:
MICOS complex subunit MIC25
Alternative name(s):
Coiled-coil-helix cristae morphology protein 1
Coiled-coil-helix-coiled-coil-helix domain-containing protein 6
Gene namesi
Name:CHCHD6
Synonyms:CHCM1, MIC25
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

HGNCiHGNC:28184. CHCHD6.

Subcellular locationi

GO - Cellular componenti

  • MICOS complex Source: UniProtKB
  • mitochondrial inner membrane Source: UniProtKB
  • mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA134899331.

Polymorphism and mutation databases

BioMutaiCHCHD6.
DMDMi62510505.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methionineiRemovedSequence analysis
Chaini2 – 235234MICOS complex subunit MIC25PRO_0000129169Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Lipidationi2 – 21N-myristoyl glycineSequence analysis
Modified residuei13 – 131PhosphoserineCombined sources
Modified residuei31 – 311PhosphoserineBy similarity
Disulfide bondi197 ↔ 228By similarity
Disulfide bondi207 ↔ 218By similarity

Keywords - PTMi

Disulfide bond, Lipoprotein, Myristate, Phosphoprotein

Proteomic databases

EPDiQ9BRQ6.
MaxQBiQ9BRQ6.
PaxDbiQ9BRQ6.
PeptideAtlasiQ9BRQ6.
PRIDEiQ9BRQ6.

PTM databases

iPTMnetiQ9BRQ6.
PhosphoSiteiQ9BRQ6.

Expressioni

Inductioni

Down-regulated following genotoxic stress.1 Publication

Gene expression databases

BgeeiQ9BRQ6.
CleanExiHS_CHCHD6.
ExpressionAtlasiQ9BRQ6. baseline and differential.
GenevisibleiQ9BRQ6. HS.

Organism-specific databases

HPAiHPA047673.
HPA051975.

Interactioni

Subunit structurei

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. 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:17624330, PubMed:22228767, PubMed:25781180, PubMed:25997101). Interacts with DISC1 (PubMed:22228767). Interacts with DISC1 (PubMed:22228767). Interacts with IMMT/MIC60 (PubMed:22228767, PubMed:25997101).4 Publications

Protein-protein interaction databases

BioGridi124029. 25 interactions.
IntActiQ9BRQ6. 15 interactions.
MINTiMINT-4830561.
STRINGi9606.ENSP00000290913.

Structurei

3D structure databases

ProteinModelPortaliQ9BRQ6.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini190 – 23142CHCHAdd
BLAST

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili129 – 17648Sequence analysisAdd
BLAST

Sequence similaritiesi

Contains 1 CHCH domain.Curated

Keywords - Domaini

Coiled coil

Phylogenomic databases

eggNOGiKOG4083. Eukaryota.
ENOG41126BR. LUCA.
GeneTreeiENSGT00390000000903.
HOGENOMiHOG000252969.
HOVERGENiHBG050936.
InParanoidiQ9BRQ6.
KOiK17564.
OrthoDBiEOG7NW6C3.
PhylomeDBiQ9BRQ6.
TreeFamiTF326279.

Family and domain databases

InterProiIPR007964. DUF737.
[Graphical view]
PfamiPF05300. DUF737. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q9BRQ6-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MGSTESSEGR RVSFGVDEEE RVRVLQGVRL SENVVNRMKE PSSPPPAPTS
60 70 80 90 100
STFGLQDGNL RAPHKESTLP RSGSSGGQQP SGMKEGVKRY EQEHAAIQDK
110 120 130 140 150
LFQVAKRERE AATKHSKASL PTGEGSISHE EQKSVRLARE LESREAELRR
160 170 180 190 200
RDTFYKEQLE RIERKNAEMY KLSSEQFHEA ASKMESTIKP RRVEPVCSGL
210 220 230
QAQILHCYRD RPHEVLLCSD LVKAYQRCVS AAHKG
Length:235
Mass (Da):26,458
Last modified:June 1, 2001 - v1
Checksum:i674DE387557AF4F0
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti95 – 951A → S.
Corresponds to variant rs2272487 [ dbSNP | Ensembl ].
VAR_024412

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
JF264889 mRNA. Translation: AEA95848.1.
AC078867 Genomic DNA. No translation available.
AC092898 Genomic DNA. No translation available.
AC117450 Genomic DNA. No translation available.
CH471052 Genomic DNA. Translation: EAW79352.1.
BC006123 mRNA. Translation: AAH06123.1.
CCDSiCCDS3041.1.
RefSeqiNP_001307539.1. NM_001320610.1.
NP_115719.1. NM_032343.2.
UniGeneiHs.518119.

Genome annotation databases

EnsembliENST00000290913; ENSP00000290913; ENSG00000159685.
GeneIDi84303.
KEGGihsa:84303.
UCSCiuc003ejf.3. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
JF264889 mRNA. Translation: AEA95848.1.
AC078867 Genomic DNA. No translation available.
AC092898 Genomic DNA. No translation available.
AC117450 Genomic DNA. No translation available.
CH471052 Genomic DNA. Translation: EAW79352.1.
BC006123 mRNA. Translation: AAH06123.1.
CCDSiCCDS3041.1.
RefSeqiNP_001307539.1. NM_001320610.1.
NP_115719.1. NM_032343.2.
UniGeneiHs.518119.

3D structure databases

ProteinModelPortaliQ9BRQ6.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124029. 25 interactions.
IntActiQ9BRQ6. 15 interactions.
MINTiMINT-4830561.
STRINGi9606.ENSP00000290913.

PTM databases

iPTMnetiQ9BRQ6.
PhosphoSiteiQ9BRQ6.

Polymorphism and mutation databases

BioMutaiCHCHD6.
DMDMi62510505.

Proteomic databases

EPDiQ9BRQ6.
MaxQBiQ9BRQ6.
PaxDbiQ9BRQ6.
PeptideAtlasiQ9BRQ6.
PRIDEiQ9BRQ6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000290913; ENSP00000290913; ENSG00000159685.
GeneIDi84303.
KEGGihsa:84303.
UCSCiuc003ejf.3. human.

Organism-specific databases

CTDi84303.
GeneCardsiCHCHD6.
HGNCiHGNC:28184. CHCHD6.
HPAiHPA047673.
HPA051975.
MIMi615634. gene.
neXtProtiNX_Q9BRQ6.
PharmGKBiPA134899331.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4083. Eukaryota.
ENOG41126BR. LUCA.
GeneTreeiENSGT00390000000903.
HOGENOMiHOG000252969.
HOVERGENiHBG050936.
InParanoidiQ9BRQ6.
KOiK17564.
OrthoDBiEOG7NW6C3.
PhylomeDBiQ9BRQ6.
TreeFamiTF326279.

Miscellaneous databases

ChiTaRSiCHCHD6. human.
GenomeRNAii84303.
PROiQ9BRQ6.
SOURCEiSearch...

Gene expression databases

BgeeiQ9BRQ6.
CleanExiHS_CHCHD6.
ExpressionAtlasiQ9BRQ6. baseline and differential.
GenevisibleiQ9BRQ6. HS.

Family and domain databases

InterProiIPR007964. DUF737.
[Graphical view]
PfamiPF05300. DUF737. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "CHCM1/CHCHD6, a novel mitochondrial protein linked to regulation of mitofilin and mitochondrial cristae morphology."
    An J., Shi J., He Q., Lui K., Liu Y., Huang Y., Sheikh M.S.
    J. Biol. Chem. 287:7411-7426(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, SUBCELLULAR LOCATION, INDUCTION, INTERACTION WITH CHCHD3; DISC1 AND IMMT.
  2. "The DNA sequence, annotation and analysis of human chromosome 3."
    Muzny D.M., Scherer S.E., Kaul R., Wang J., Yu J., Sudbrak R., Buhay C.J., Chen R., Cree A., Ding Y., Dugan-Rocha S., Gill R., Gunaratne P., Harris R.A., Hawes A.C., Hernandez J., Hodgson A.V., Hume J.
    , Jackson A., Khan Z.M., Kovar-Smith C., Lewis L.R., Lozado R.J., Metzker M.L., Milosavljevic A., Miner G.R., Morgan M.B., Nazareth L.V., Scott G., Sodergren E., Song X.-Z., Steffen D., Wei S., Wheeler D.A., Wright M.W., Worley K.C., Yuan Y., Zhang Z., Adams C.Q., Ansari-Lari M.A., Ayele M., Brown M.J., Chen G., Chen Z., Clendenning J., Clerc-Blankenburg K.P., Chen R., Chen Z., Davis C., Delgado O., Dinh H.H., Dong W., Draper H., Ernst S., Fu G., Gonzalez-Garay M.L., Garcia D.K., Gillett W., Gu J., Hao B., Haugen E., Havlak P., He X., Hennig S., Hu S., Huang W., Jackson L.R., Jacob L.S., Kelly S.H., Kube M., Levy R., Li Z., Liu B., Liu J., Liu W., Lu J., Maheshwari M., Nguyen B.-V., Okwuonu G.O., Palmeiri A., Pasternak S., Perez L.M., Phelps K.A., Plopper F.J., Qiang B., Raymond C., Rodriguez R., Saenphimmachak C., Santibanez J., Shen H., Shen Y., Subramanian S., Tabor P.E., Verduzco D., Waldron L., Wang J., Wang J., Wang Q., Williams G.A., Wong G.K.-S., Yao Z., Zhang J., Zhang X., Zhao G., Zhou J., Zhou Y., Nelson D., Lehrach H., Reinhardt R., Naylor S.L., Yang H., Olson M., Weinstock G., Gibbs R.A.
    Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Lung.
  5. "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."
    Xie J., Marusich M.F., Souda P., Whitelegge J., Capaldi R.A.
    FEBS Lett. 581:3545-3549(2007) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION IN A COMPLEX WITH IMMT.
  6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  8. Cited for: NOMENCLATURE.
  9. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-13, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  10. "QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology."
    Guarani V., McNeill E.M., Paulo J.A., Huttlin E.L., Froehlich F., Gygi S.P., Van Vactor D., Harper J.W.
    Elife 4:0-0(2015) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION IN THE MICOS COMPLEX, INTERACTION WITH IMMT, SUBCELLULAR LOCATION.
  11. "Detailed analysis of the human mitochondrial contact site complex indicate a hierarchy of subunits."
    Ott C., Dorsch E., Fraunholz M., Straub S., Kozjak-Pavlovic V.
    PLoS ONE 10:E0120213-E0120213(2015) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION IN THE MICOS COMPLEX, SUBCELLULAR LOCATION.
  12. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiMIC25_HUMAN
AccessioniPrimary (citable) accession number: Q9BRQ6
Secondary accession number(s): D6R9U0, D6RIB4, H8Y0Y7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: April 12, 2005
Last sequence update: June 1, 2001
Last modified: June 8, 2016
This is version 100 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.