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Protein

MICOS complex subunit MIC25

Gene

CHCHD6

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Enzyme and pathway databases

ReactomeiR-HSA-8949613 Cristae formation

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

EuPathDBiHostDB:ENSG00000159685.10
HGNCiHGNC:28184 CHCHD6
MIMi615634 gene
neXtProtiNX_Q9BRQ6

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi84303
OpenTargetsiENSG00000159685
PharmGKBiPA134899331

Polymorphism and mutation databases

BioMutaiCHCHD6
DMDMi62510505

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedSequence analysis
ChainiPRO_00001291692 – 235MICOS complex subunit MIC25Add BLAST234

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Lipidationi2N-myristoyl glycineSequence analysis1
Modified residuei13PhosphoserineCombined sources1
Modified residuei31PhosphoserineBy similarity1
Disulfide bondi197 ↔ 228PROSITE-ProRule annotation
Disulfide bondi207 ↔ 218PROSITE-ProRule annotation

Keywords - PTMi

Disulfide bond, Lipoprotein, Myristate, Phosphoprotein

Proteomic databases

EPDiQ9BRQ6
MaxQBiQ9BRQ6
PaxDbiQ9BRQ6
PeptideAtlasiQ9BRQ6
PRIDEiQ9BRQ6
ProteomicsDBi78807

PTM databases

iPTMnetiQ9BRQ6
PhosphoSitePlusiQ9BRQ6

Expressioni

Inductioni

Down-regulated following genotoxic stress.1 Publication

Gene expression databases

BgeeiENSG00000159685 Expressed in 160 organ(s), highest expression level in anterior cingulate cortex
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 and MTX2 (together described as components of the mitochondrial outer membrane sorting assembly machinery (SAM) complex) and DNAJC11, mitochondrial inner membrane protein TMEM11 and with HSPA9 (PubMed:17624330, PubMed:22228767, PubMed:25781180, PubMed:25997101). The MICOS and SAM complexes together with DNAJC11 are part of a large protein complex spanning both membranes termed the mitochondrial intermembrane space bridging (MIB) complex. Interacts with DISC1 (PubMed:22228767). Interacts with DISC1 (PubMed:22228767). Interacts with IMMT/MIC60 (PubMed:22228767, PubMed:25997101).Curated4 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
CHCHD10Q8WYQ32EBI-2557895,EBI-16721660

Protein-protein interaction databases

BioGridi124029, 32 interactors
CORUMiQ9BRQ6
IntActiQ9BRQ6, 24 interactors
MINTiQ9BRQ6
STRINGi9606.ENSP00000290913

Structurei

3D structure databases

ProteinModelPortaliQ9BRQ6
SMRiQ9BRQ6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini194 – 235CHCHPROSITE-ProRule annotationAdd BLAST42

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili129 – 176Sequence analysisAdd BLAST48

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi197 – 207Cx9C motif 1PROSITE-ProRule annotationAdd BLAST11
Motifi218 – 228Cx9C motif 2PROSITE-ProRule annotationAdd BLAST11

Sequence similaritiesi

Keywords - Domaini

Coiled coil

Phylogenomic databases

eggNOGiKOG4083 Eukaryota
ENOG41126BR LUCA
GeneTreeiENSGT00390000000903
HOGENOMiHOG000252969
HOVERGENiHBG050936
InParanoidiQ9BRQ6
KOiK17564
PhylomeDBiQ9BRQ6
TreeFamiTF326279

Family and domain databases

InterProiView protein in InterPro
IPR007964 MIC19/MIC25
PfamiView protein in Pfam
PF05300 DUF737, 1 hit
PROSITEiView protein in PROSITE
PS51808 CHCH, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 3 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
J3QTA6J3QTA6_HUMAN
MICOS complex subunit
CHCHD6
256Annotation score:
H0Y922H0Y922_HUMAN
MICOS complex subunit
CHCHD6
166Annotation score:
J3QTB2J3QTB2_HUMAN
MICOS complex subunit
CHCHD6
140Annotation score:

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02441295A → S. Corresponds to variant dbSNP:rs2272487Ensembl.1

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

Similar proteinsi

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
SMRiQ9BRQ6
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124029, 32 interactors
CORUMiQ9BRQ6
IntActiQ9BRQ6, 24 interactors
MINTiQ9BRQ6
STRINGi9606.ENSP00000290913

PTM databases

iPTMnetiQ9BRQ6
PhosphoSitePlusiQ9BRQ6

Polymorphism and mutation databases

BioMutaiCHCHD6
DMDMi62510505

Proteomic databases

EPDiQ9BRQ6
MaxQBiQ9BRQ6
PaxDbiQ9BRQ6
PeptideAtlasiQ9BRQ6
PRIDEiQ9BRQ6
ProteomicsDBi78807

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi84303
DisGeNETi84303
EuPathDBiHostDB:ENSG00000159685.10
GeneCardsiCHCHD6
HGNCiHGNC:28184 CHCHD6
HPAiHPA047673
HPA051975
MIMi615634 gene
neXtProtiNX_Q9BRQ6
OpenTargetsiENSG00000159685
PharmGKBiPA134899331
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4083 Eukaryota
ENOG41126BR LUCA
GeneTreeiENSGT00390000000903
HOGENOMiHOG000252969
HOVERGENiHBG050936
InParanoidiQ9BRQ6
KOiK17564
PhylomeDBiQ9BRQ6
TreeFamiTF326279

Enzyme and pathway databases

ReactomeiR-HSA-8949613 Cristae formation

Miscellaneous databases

ChiTaRSiCHCHD6 human
GenomeRNAii84303
PROiPR:Q9BRQ6
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000159685 Expressed in 160 organ(s), highest expression level in anterior cingulate cortex
CleanExiHS_CHCHD6
ExpressionAtlasiQ9BRQ6 baseline and differential
GenevisibleiQ9BRQ6 HS

Family and domain databases

InterProiView protein in InterPro
IPR007964 MIC19/MIC25
PfamiView protein in Pfam
PF05300 DUF737, 1 hit
PROSITEiView protein in PROSITE
PS51808 CHCH, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiMIC25_HUMAN
AccessioniPrimary (citable) accession number: Q9BRQ6
Secondary accession number(s): D6R9U0, D6RIB4, H8Y0Y7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 12, 2005
Last sequence update: June 1, 2001
Last modified: October 10, 2018
This is version 118 of the entry and version 1 of the sequence. See 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. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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