CHCHD4

Gene Information

Official Symbol: CHCHD4
Official Name: coiled-coil-helix-coiled-coil-helix domain containing 4
Aliases and Previous Symbols: N/A
Entrez ID: 131474
UniProt: Q8N4Q1
Interactions: BioGRID
PubMed articles: Open PubMed
OMIM: Open OMIM

Function Summary

Entrez Summary: N/A
UniProt Summary: Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364). Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS) (PubMed:16185709, PubMed:19182799, PubMed:21059946). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria (PubMed:16185709, PubMed:19182799, PubMed:21059946). 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). 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 heterodimer that regulates mitochondrial calcium uptake (PubMed:26387864). {ECO:0000269|PubMed:16185709, ECO:0000269|PubMed:19182799, ECO:0000269|PubMed:21059946, ECO:0000269|PubMed:23186364, ECO:0000269|PubMed:26387864}.

Pfam Domains GO Terms

CRISPR Data

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Screen	Score
MS023 7μM R03 exp135	-2.1
Nocodazole 0.02μM R00 exp23	-1.83
Citral 50μM R06 exp274	-1.82
BRD2 inhibitor II 20μM R07 exp341	-1.72
PF-06409577 20μM R07 exp389	-1.71
JQ1 0.8μM R08 exp435	1.75
SGC0946 7μM R03 exp151	1.84
PU-H71 1μM R08 exp515	1.95
ONC212 0.15μM R08 exp513	2.03
GSK-J4 1.5μM R08 exp441	2.13
Tanespimycin 14μM R08 exp527	2.71

Gene	Correlation
TRIAP1	0.564
MRPS34	0.56
MRPL21	0.553
GMPPB	0.55
SUPV3L1	0.548
RPIA	0.546
HARS	0.542
HSD17B10	0.536
PMM2	0.536
MRP63	0.532
THG1L	0.532
SRP14	0.53
SARS2	0.53
MRPL15	0.529
RPUSD4	0.527
YARS2	0.523
MRPS18A	0.523
MRPL23	0.521
UAP1	0.52
PDSS1	0.52
FASTKD5	0.519
MRPS5	0.515
DHRSX	0.512
ALG13	0.511
BCS1L	0.508
PPA2	0.507
TEN1	0.507
AASDHPPT	0.505
MRPS2	0.503
MRPS14	0.502
MRPS6	0.502
DPAGT1	0.501
TAZ	0.5
UQCR10	0.5
LSM10	0.499
MRPS33	0.496
MRPL53	0.496
VEZT	0.494
TAMM41	0.493
TUFM	0.492
GFM1	0.49
GEMIN7	0.489
MRPS9	0.488
COX16	0.488
TARS2	0.487
ADSL	0.487
RNASEH1	0.485
DARS2	0.485
UHRF1	0.483
SLC25A26	0.482
C17orf89	0.48
ATXN10	0.477
EIF3F	0.476
MRPS16	0.475
RPE	0.474
MRPS23	0.473
ATIC	0.472
MRPS24	0.472
PIK3R4	0.472
MRPS26	0.471
DAP3	0.471
GTF3C6	0.471
KIAA0391	0.47
C12orf65	0.47
PPCS	0.469
TXNL4B	0.468
SCO2	0.468
POLG	0.467
FH	0.467
ELAC2	0.467
ALG1	0.466
HCCS	0.465
TRNT1	0.465
RBFA	0.465
EARS2	0.461
MRPS11	0.461
PTDSS1	0.461
MRPS21	0.459
CBFB	0.459
GEMIN8	0.454
MTPAP	0.454
NAE1	0.451
C10orf2	0.451
SELRC1	0.451
TPK1	0.45
TMA16	0.45
RABGGTB	0.45
NBN	0.449
COX5A	0.449
NDUFA3	0.447
PGGT1B	0.446
COX6C	0.445
RBMX2	0.444
MRPL47	0.443
TMEM242	0.442
HAUS8	0.441
ALG8	0.441
NDUFB5	0.44
GFPT1	0.44
PARS2	0.44
RNMT	0.44
UQCRC2	0.44
GEMIN6	0.44
MTG2	0.439
ALG6	0.438
RPTOR	0.437
GTPBP10	0.436
SDHA	0.435
FNTA	0.434
TEFM	0.434
DHPS	0.433
VARS2	0.432
DOHH	0.431
MRPL34	0.43
ATP5D	0.429
SEC62	0.428
STT3A	0.427
ATP5J	0.427
BAP1	0.426
PGM3	0.425
TOMM40	0.423
GTF3C4	0.423
RPP21	0.421
DDX20	0.421
ATP5B	0.417
UQCRQ	0.417
LSM11	0.416
NDUFB1	0.416
GTF2A2	0.416
TIMM8B	0.416
MRPL12	0.415
UQCC2	0.412
SEC61G	0.411
RTCB	0.411
SLC25A19	0.411
DHX30	0.41
COX17	0.41
GTF3C3	0.41
RAD1	0.41
DOLK	0.41
UTP23	0.409
TMEM126B	0.409
KDSR	0.409
NDUFAF4	0.408
ROMO1	0.408
PELO	0.407
TRMT61A	0.407
MBTPS2	0.406
UXT	0.406
MRPL20	0.405
YARS	0.405
TPRKB	0.404
MTOR	0.404
CBX3	0.403
COX7B	0.403
COA6	0.403
PPP4C	0.403
MRPL55	0.402
TTC4	0.402
NSMCE1	0.402
CRLS1	0.4

Global Fraction of Cell Lines Where Essential: 471/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	21/28
blood	7/28
bone	19/26
breast	14/33
central nervous system	39/56
cervix	4/4
colorectal	12/17
esophagus	9/13
fibroblast	0/1
gastric	10/16
kidney	16/21
liver	14/20
lung	53/75
lymphocyte	5/16
ovary	15/26
pancreas	18/24
peripheral nervous system	12/16
plasma cell	4/15
prostate	1/1
skin	14/24
soft tissue	5/9
thyroid	1/2
upper aerodigestive	20/22
urinary tract	20/29
uterus	3/5

Essentiality in NALM6

Essentiality Rank: 7667
Expression level (log2 read counts): 4.2

Expression Distribution

protein import into mitochondrial intermembrane space
peptidyl-cysteine oxidation
protein maturation by protein folding
protein oxidation
protein disulfide oxidoreductase activity
protein transmembrane import into intracellular organelle
de novo posttranslational protein folding
peptidyl-cysteine modification
de novo protein folding
intracellular protein transmembrane transport
protein targeting to mitochondrion
protein transmembrane transport
establishment of protein localization to mitochondrion
protein localization to mitochondrion
mitochondrial intermembrane space
mitochondrial transmembrane transport
protein import
mitochondrial transport
protein maturation
protein folding
protein targeting
establishment of protein localization to organelle
mitochondrion organization
protein localization to organelle
peptidyl-amino acid modification
oxidation-reduction process
intracellular protein transport
mitochondrion
transmembrane transport
protein transport
intracellular transport
peptide transport
amide transport
cellular protein localization
cellular macromolecule localization
establishment of protein localization
establishment of localization in cell
nitrogen compound transport
gene expression