mitochondrial intermembrane space Gene Set

Dataset GO Cellular Component Annotations 2025
Category structural or functional annotations
Type cellular component
Description The region between the inner and outer lipid bilayers of the mitochondrial envelope. (Gene Ontology, GO_0005758)
External Link http://amigo.geneontology.org/amigo/term/GO:0005758
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Genes

72 proteins localized to the mitochondrial intermembrane space cellular component from the curated GO Cellular Component Annotations 2025 dataset.

Symbol Name
AGK acylglycerol kinase
AIFM1 apoptosis-inducing factor, mitochondrion-associated, 1
AK2 adenylate kinase 2
AKT1 v-akt murine thymoma viral oncogene homolog 1
ALPL alkaline phosphatase, liver/bone/kidney
ARL2 ADP-ribosylation factor-like 2
ARL2BP ADP-ribosylation factor-like 2 binding protein
BLOC1S1 biogenesis of lysosomal organelles complex-1, subunit 1
CD163L1 CD163 molecule-like 1
CEP89 centrosomal protein 89kDa
CHCHD10 coiled-coil-helix-coiled-coil-helix domain containing 10
CHCHD2 coiled-coil-helix-coiled-coil-helix domain containing 2
CHCHD4 coiled-coil-helix-coiled-coil-helix domain containing 4
CHCHD5 coiled-coil-helix-coiled-coil-helix domain containing 5
CLPB ClpB homolog, mitochondrial AAA ATPase chaperonin
CMC4 C-x(9)-C motif containing 4
COA4 cytochrome c oxidase assembly factor 4 homolog (S. cerevisiae)
COA6 cytochrome c oxidase assembly factor 6
COA7 cytochrome c oxidase assembly factor 7 (putative)
COX17 COX17 cytochrome c oxidase copper chaperone
COX19 COX19 cytochrome c oxidase assembly factor
COX4I1 cytochrome c oxidase subunit IV isoform 1
COX4I2 cytochrome c oxidase subunit IV isoform 2 (lung)
COX5A cytochrome c oxidase subunit Va
COX6C cytochrome c oxidase subunit VIc
CPOX coproporphyrinogen oxidase
CYCS cytochrome c, somatic
DIABLO diablo, IAP-binding mitochondrial protein
FBXL4 F-box and leucine-rich repeat protein 4
GATM glycine amidinotransferase (L-arginine:glycine amidinotransferase)
HAX1 HCLS1 associated protein X-1
HSD3B1 hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 1
HSD3B2 hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2
HTRA2 HtrA serine peptidase 2
MED12 mediator complex subunit 12
MICU1 mitochondrial calcium uptake 1
MICU2 mitochondrial calcium uptake 2
MYOC myocilin, trabecular meshwork inducible glucocorticoid response
NDUFA8 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa
NDUFB7 NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa
NDUFS1 NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)
NLN neurolysin (metallopeptidase M3 family)
NME4 NME/NM23 nucleoside diphosphate kinase 4
OMA1 OMA1 zinc metallopeptidase
PANK2 pantothenate kinase 2
PINK1 PTEN induced putative kinase 1
PNPT1 polyribonucleotide nucleotidyltransferase 1
PPOX protoporphyrinogen oxidase
PRELID1 PRELI domain containing 1
PRELID2 PRELI domain containing 2
PRELID3A PRELI domain containing 3A
PRELID3B PRELI domain containing 3B
REXO2 RNA exonuclease 2
SDHAF3 succinate dehydrogenase complex assembly factor 3
SIRT5 sirtuin 5
SOD1 superoxide dismutase 1, soluble
STMP1 short transmembrane mitochondrial protein 1
STOML2 stomatin (EPB72)-like 2
TAFAZZIN tafazzin, phospholipid-lysophospholipid transacylase
THOP1 thimet oligopeptidase 1
TIMM10 translocase of inner mitochondrial membrane 10 homolog (yeast)
TIMM10B translocase of inner mitochondrial membrane 10 homolog B (yeast)
TIMM13 translocase of inner mitochondrial membrane 13 homolog (yeast)
TIMM21 translocase of inner mitochondrial membrane 21 homolog (yeast)
TIMM23 translocase of inner mitochondrial membrane 23 homolog (yeast)
TIMM29 translocase of inner mitochondrial membrane 29
TIMM8A translocase of inner mitochondrial membrane 8 homolog A (yeast)
TIMM8B translocase of inner mitochondrial membrane 8 homolog B (yeast)
TIMM9 translocase of inner mitochondrial membrane 9 homolog (yeast)
TRAP1 TNF receptor-associated protein 1
TRIAP1 TP53 regulated inhibitor of apoptosis 1
UQCC2 ubiquinol-cytochrome c reductase complex assembly factor 2