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Tbio
CHCHD4
Mitochondrial intermembrane space import and assembly protein 40

Protein Summary
Description
Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364, PubMed:23676665). Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS (PubMed:16185709, PubMed:19182799, PubMed:21059946, PubMed:23676665). Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system (PubMed:23186364). Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 ...more
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000295767
  • ENSP00000295767
  • ENSG00000163528
  • ENST00000396914
  • ENSP00000380122

Symbol
  • MIA40
  • MIA40
  • TIMM40
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
histone modification site profile
0.83
transcription factor binding site profile
0.77
transcription factor perturbation
0.68
cell type or tissue
0.67
tissue sample
0.65


Related Tools
Target Illumination GWAS Analytics (TIGA)
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TIGA scores and ranks GWAS discovered associations according to the quantity and quality of the evidence supporting the association.
GENEVA
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GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
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GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
IDG Development Level Summary
Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 101.85   (req: < 5)
Gene RIFs: 17   (req: <= 3)
Antibodies: 157   (req: <= 50)
Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 101.85   (req: >= 5)
Gene RIFs: 17   (req: > 3)
Antibodies: 157   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 5
Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0
Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0
GWAS Traits (7)
GWAS Trait
EFO ID
Study Count
SNP Count
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
IGF-1 measurement
1
1
1
47.7
heel bone mineral density
1
1
1
30.2
BMI-adjusted hip circumference
1
1
1
21.5
longevity
1
1
1
15.8
monocyte percentage of leukocytes
1
1
1
14.2
GWAS Trait
EFO ID
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
IGF-1 measurement
1
47.7
heel bone mineral density
1
30.2
BMI-adjusted hip circumference
1
21.5
longevity
1
15.8
monocyte percentage of leukocytes
1
14.2
Orthologs (14)
1 – 5 of 14
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
coiled-coil-helix-coiled-coil-helix domain containing 4
VGNC:7964
470755
Mouse
MGI:1919420
72170
Rat
Dog
coiled-coil-helix-coiled-coil-helix domain containing 4
VGNC:39196
476520
Horse
coiled-coil-helix-coiled-coil-helix domain containing 4 [Source:HGNC Symbol;Acc:HGNC:26467]
Species
Name
OMA
EggNOG
Inparanoid
Chimp
coiled-coil-helix-coiled-coil-helix domain containing 4
Mouse
Rat
Dog
coiled-coil-helix-coiled-coil-helix domain containing 4
Horse
coiled-coil-helix-coiled-coil-helix domain containing 4 [Source:HGNC Symbol;Acc:HGNC:26467]
Protein Structure (2 Structures, 1 AlphaFold Model)
RepresentationColor Scheme

Click on a row in the table to change the structure displayed.
More information can be found at RCSB PDB

AF-Q8N4Q1-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

1 – 2 of 2
PDB Structure Id
Ligand
Method
Resolution (Å)
Residues
Fraction of Total Protein
Pub Year
Title
PDB Structure Id
Fraction of Total Protein
Resolution
Pub Year
Pathways (2)
Mitochondrial protein import (R-HSA-1268020)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 2 of 2
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Mitochondrial protein import
Reactome
Protein localization
Name
Explore in Pharos
Explore in Source
Mitochondrial protein import
Protein localization
Gene Ontology Terms (7)
Items per page:
10
1 – 1 of 1
GO Term
Evidence
Assigned by
Inferred from Direct Assay (IDA)
UniProtKB
Protein-Protein Interactions (169)
1 – 10 of 169
RPS6KA5
Tchem
Family:  Kinase
Novelty:  0.00811496
p_int:  0.999999996
p_ni:  4e-9
Data Source:  BioPlex
MICAL3
Tbio
Family:  Enzyme
Novelty:  0.13432068
p_int:  0.999999993
p_ni:  2e-9
p_wrong:  4e-9
Data Source:  BioPlex
ZBTB10
Tbio
Family:  TF
Novelty:  0.05707286
p_int:  0.99999992
p_ni:  8e-8
Data Source:  BioPlex
COA5
Tdark
Family:  Enzyme
Novelty:  0.1358881
p_int:  0.999999899
p_ni:  1e-7
p_wrong:  1e-9
Score:  0.854
Data Source:  BioPlex,STRINGDB
ZNF581
Tdark
Family:  TF
Novelty:  18
p_int:  0.999999212
p_ni:  1e-9
p_wrong:  7.87e-7
Data Source:  BioPlex
GFER
Tchem
Family:  Enzyme
Novelty:  0.00338563
p_int:  0.99999918
p_ni:  8.2e-7
Score:  0.997
Data Source:  BioPlex,Reactome,STRINGDB
FAM193A
Tdark
Novelty:  0.35264727
p_int:  0.999994242
p_ni:  0.000005631
p_wrong:  1.27e-7
Data Source:  BioPlex
KLHDC3
Tdark
Novelty:  0.63840156
p_int:  0.999987355
p_ni:  0.000012644
p_wrong:  1e-9
Data Source:  BioPlex
MED29
Tbio
Family:  Enzyme
Novelty:  0.01740647
p_int:  0.999983473
p_ni:  0.000016527
Data Source:  BioPlex
CHCHD5
Tbio
Novelty:  0.16304141
p_int:  0.999978993
p_ni:  0.000017163
p_wrong:  0.000003844
Score:  0.955
Data Source:  BioPlex,STRINGDB
Publication Statistics
PubMed Score  101.85

PubMed score by year
PubTator Score  55.3

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer
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