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Protein

MICOS complex subunit MIC19

Gene

CHCHD3

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. 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).2 Publications

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionRepressor
Biological processTranscription, Transcription regulation

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import
R-HSA-8949613 Cristae formation

Names & Taxonomyi

Protein namesi
Recommended name:
MICOS complex subunit MIC19
Alternative name(s):
Coiled-coil-helix-coiled-coil-helix domain-containing protein 3
Gene namesi
Name:CHCHD3
Synonyms:MIC19, MINOS3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

EuPathDBiHostDB:ENSG00000106554.11
HGNCiHGNC:21906 CHCHD3
MIMi613748 gene
neXtProtiNX_Q9NX63

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

Cytoplasm, Membrane, Mitochondrion, Mitochondrion inner membrane, Nucleus

Pathology & Biotechi

Organism-specific databases

DisGeNETi54927
OpenTargetsiENSG00000106554
PharmGKBiPA134983108

Polymorphism and mutation databases

BioMutaiCHCHD3
DMDMi62510520

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemoved1 Publication
ChainiPRO_00001291632 – 227MICOS complex subunit MIC19Add BLAST226

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Lipidationi2N-myristoyl glycine1 Publication1
Modified residuei29PhosphoserineCombined sources1
Modified residuei49PhosphotyrosineCombined sources1
Modified residuei50PhosphoserineCombined sources1
Modified residuei56PhosphoserineBy similarity1
Modified residuei58PhosphoserineCombined sources1
Modified residuei142N6-acetyllysineCombined sources1
Disulfide bondi183 ↔ 214PROSITE-ProRule annotation
Disulfide bondi193 ↔ 204PROSITE-ProRule annotation

Keywords - PTMi

Acetylation, Disulfide bond, Lipoprotein, Myristate, Phosphoprotein

Proteomic databases

EPDiQ9NX63
MaxQBiQ9NX63
PaxDbiQ9NX63
PeptideAtlasiQ9NX63
PRIDEiQ9NX63
ProteomicsDBi83048
TopDownProteomicsiQ9NX63

2D gel databases

REPRODUCTION-2DPAGEiIPI00015833
UCD-2DPAGEiQ9NX63

PTM databases

iPTMnetiQ9NX63
PhosphoSitePlusiQ9NX63
SwissPalmiQ9NX63

Expressioni

Tissue specificityi

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.1 Publication

Gene expression databases

BgeeiENSG00000106554 Expressed in 127 organ(s), highest expression level in quadriceps femoris
CleanExiHS_CHCHD3
ExpressionAtlasiQ9NX63 baseline and differential
GenevisibleiQ9NX63 HS

Organism-specific databases

HPAiHPA042935

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 (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 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:21081504, PubMed:22114354, PubMed:22228767, 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 HSPA1A/HSPA1B and OPA1, preferentially with the soluble OPA1 form (By similarity). Interacts with IMMT/MIC60.By similarity6 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi120267, 88 interactors
CORUMiQ9NX63
IntActiQ9NX63, 101 interactors
MINTiQ9NX63
STRINGi9606.ENSP00000262570

Structurei

3D structure databases

ProteinModelPortaliQ9NX63
SMRiQ9NX63
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini180 – 222CHCHPROSITE-ProRule annotationAdd BLAST43

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi183 – 193Cx9C motif 1PROSITE-ProRule annotationAdd BLAST11
Motifi204 – 214Cx9C motif 2PROSITE-ProRule annotationAdd BLAST11

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG4083 Eukaryota
ENOG41126BR LUCA
GeneTreeiENSGT00390000000903
HOGENOMiHOG000252969
HOVERGENiHBG050936
InParanoidiQ9NX63
KOiK17563
PhylomeDBiQ9NX63
TreeFamiTF326279

Family and domain databases

InterProiView protein in InterPro
IPR007964 MIC19/MIC25
PfamiView protein in Pfam
PF05300 DUF737, 2 hits
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

Q9NX63-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MGGTTSTRRV TFEADENENI TVVKGIRLSE NVIDRMKESS PSGSKSQRYS
60 70 80 90 100
GAYGASVSDE ELKRRVAEEL ALEQAKKESE DQKRLKQAKE LDRERAAANE
110 120 130 140 150
QLTRAILRER ICSEEERAKA KHLARQLEEK DRVLKKQDAF YKEQLARLEE
160 170 180 190 200
RSSEFYRVTT EQYQKAAEEV EAKFKRYESH PVCADLQAKI LQCYRENTHQ
210 220
TLKCSALATQ YMHCVNHAKQ SMLEKGG
Length:227
Mass (Da):26,152
Last modified:October 1, 2000 - v1
Checksum:i569E405EB7C801D0
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F8WAR4F8WAR4_HUMAN
MICOS complex subunit
CHCHD3
241Annotation score:
A0A286YEX5A0A286YEX5_HUMAN
MICOS complex subunit
CHCHD3
101Annotation score:
C9JRZ6C9JRZ6_HUMAN
MICOS complex subunit
CHCHD3
232Annotation score:

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK000427 mRNA Translation: BAA91157.1
BC011596 mRNA Translation: AAH11596.1
BC014839 mRNA Translation: AAH14839.1
CCDSiCCDS5828.1
RefSeqiNP_001304106.1, NM_001317177.1
NP_060282.1, NM_017812.3
UniGeneiHs.655010

Genome annotation databases

EnsembliENST00000262570; ENSP00000262570; ENSG00000106554
GeneIDi54927
KEGGihsa:54927
UCSCiuc003vre.4 human

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK000427 mRNA Translation: BAA91157.1
BC011596 mRNA Translation: AAH11596.1
BC014839 mRNA Translation: AAH14839.1
CCDSiCCDS5828.1
RefSeqiNP_001304106.1, NM_001317177.1
NP_060282.1, NM_017812.3
UniGeneiHs.655010

3D structure databases

ProteinModelPortaliQ9NX63
SMRiQ9NX63
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi120267, 88 interactors
CORUMiQ9NX63
IntActiQ9NX63, 101 interactors
MINTiQ9NX63
STRINGi9606.ENSP00000262570

PTM databases

iPTMnetiQ9NX63
PhosphoSitePlusiQ9NX63
SwissPalmiQ9NX63

Polymorphism and mutation databases

BioMutaiCHCHD3
DMDMi62510520

2D gel databases

REPRODUCTION-2DPAGEiIPI00015833
UCD-2DPAGEiQ9NX63

Proteomic databases

EPDiQ9NX63
MaxQBiQ9NX63
PaxDbiQ9NX63
PeptideAtlasiQ9NX63
PRIDEiQ9NX63
ProteomicsDBi83048
TopDownProteomicsiQ9NX63

Protocols and materials databases

DNASUi54927
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000262570; ENSP00000262570; ENSG00000106554
GeneIDi54927
KEGGihsa:54927
UCSCiuc003vre.4 human

Organism-specific databases

CTDi54927
DisGeNETi54927
EuPathDBiHostDB:ENSG00000106554.11
GeneCardsiCHCHD3
HGNCiHGNC:21906 CHCHD3
HPAiHPA042935
MIMi613748 gene
neXtProtiNX_Q9NX63
OpenTargetsiENSG00000106554
PharmGKBiPA134983108
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4083 Eukaryota
ENOG41126BR LUCA
GeneTreeiENSGT00390000000903
HOGENOMiHOG000252969
HOVERGENiHBG050936
InParanoidiQ9NX63
KOiK17563
PhylomeDBiQ9NX63
TreeFamiTF326279

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import
R-HSA-8949613 Cristae formation

Miscellaneous databases

ChiTaRSiCHCHD3 human
GenomeRNAii54927
PROiPR:Q9NX63
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000106554 Expressed in 127 organ(s), highest expression level in quadriceps femoris
CleanExiHS_CHCHD3
ExpressionAtlasiQ9NX63 baseline and differential
GenevisibleiQ9NX63 HS

Family and domain databases

InterProiView protein in InterPro
IPR007964 MIC19/MIC25
PfamiView protein in Pfam
PF05300 DUF737, 2 hits
PROSITEiView protein in PROSITE
PS51808 CHCH, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiMIC19_HUMAN
AccessioniPrimary (citable) accession number: Q9NX63
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 12, 2005
Last sequence update: October 1, 2000
Last modified: November 7, 2018
This is version 148 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. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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