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NDUFS1 [mitochondrial matrix]
Stable Identifier
R-HSA-6788513
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
NADH-ubiquinone oxidoreductase 75 kDa subunit
Locations in the PathwayBrowser
Expand all
Metabolism (Homo sapiens)
The citric acid (TCA) cycle and respiratory electron transport (Homo sapiens)
Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. (Homo sapiens)
Respiratory electron transport (Homo sapiens)
Complex I biogenesis (Homo sapiens)
NDUF subunits bind to form the FP subcomplex (Homo sapiens)
FP subcomplex [mitochondrial inner membrane] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
NUBPL transfers 4Fe-4S to Complex I subunits (Homo sapiens)
NDUF subunits [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
NDUF:4Fe-4S subunits [mitochondrial matrix] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
Peripheral arm subunits bind the 815kDa complex to form a 980kDa complex (Homo sapiens)
980kDa complex [mitochondrial inner membrane] (Homo sapiens)
FP subcomplex [mitochondrial inner membrane] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
FP subcomplex [mitochondrial inner membrane] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
The MCIA complex, NDUFAF2-7 all dissociate from the 980kDa complex, resulting in Complex I (Homo sapiens)
980kDa complex [mitochondrial inner membrane] (Homo sapiens)
FP subcomplex [mitochondrial inner membrane] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
Complex I [mitochondrial inner membrane] (Homo sapiens)
FP subcomplex (-NDUFAF2) [mitochondrial inner membrane] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
Complex I oxidises NADH to NAD+, reduces CoQ to QH2 (Homo sapiens)
Complex I [mitochondrial inner membrane] (Homo sapiens)
FP subcomplex (-NDUFAF2) [mitochondrial inner membrane] (Homo sapiens)
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
Metabolism of proteins (Homo sapiens)
Mitochondrial protein degradation (Homo sapiens)
CLPXP binds mitochondrial matrix proteins (Homo sapiens)
CLPXP:substrate protein (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
CLPXP degrades mitochondrial matrix proteins (Homo sapiens)
CLPXP:substrate protein (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
LONP1 binds mitochondrial matrix proteins (Homo sapiens)
LONP1:substrate protein (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of LONP1 (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
Substrate of LONP1 (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
LONP1 degrades mitochondrial matrix proteins (Homo sapiens)
LONP1:substrate protein (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of LONP1 (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUFS1 [mitochondrial matrix] (Homo sapiens)
External Reference Information
External Reference
UniProt:P28331 NDUFS1
Gene Names
NDUFS1
Chain
transit peptide:1-23, chain:24-727
Reference Genes
BioGPS Gene:4719 NDUFS1
COSMIC (genes):NDUFS1 NDUFS1
CTD Gene:4719 NDUFS1
dbSNP Gene:4719 NDUFS1
ENSEMBL:ENSG00000023228 NDUFS1
ENSEMBL_homo_sapiens_GENE:ENSG00000023228.14 NDUFS1
ENSEMBL_homo_sapiens_GENE:ENSG00000283447.2 NDUFS1
HGNC:7707 NDUFS1
KEGG Gene (Homo sapiens):4719 NDUFS1
Monarch:4719 NDUFS1
NCBI Gene:4719 NDUFS1
OMIM:157655 NDUFS1
UCSC:P28331 NDUFS1
Reference Transcript
RefSeq:NM_001199983.1 NDUFS1
RefSeq:NM_001199981.1 NDUFS1
RefSeq:NM_001199982.1 NDUFS1
RefSeq:NM_001199984.1 NDUFS1
RefSeq:NM_005006.6 NDUFS1
Other Identifiers
11731416_at
11731417_a_at
11744901_s_at
11749697_a_at
11749722_a_at
11757936_s_at
16907546
16907548
203039_PM_s_at
203039_s_at
229647_PM_at
229647_at
235321_PM_at
235321_at
236356_PM_at
236356_at
2442901
2442902
2442903
2596198
2596199
2596200
2596202
2596203
2596204
2596205
2596206
2596207
2596208
2596209
2596210
2596211
2596212
2596213
2596214
2596215
2596216
2596217
2596219
2596220
2596222
2596223
2596224
2596225
2596227
2596228
2596229
2596230
2596231
2596232
2596233
2596234
2596235
2596236
2596241
2596242
2596244
2596247
2851484
3701463
3701464
3701466
38395_at
43622_at
4719
53769_at
55695_at
59297_at
8058428
A_23_P131363
A_23_P131365
A_24_P696507
GE59867
GE88074
GO:0003824
GO:0005215
GO:0005515
GO:0005739
GO:0005743
GO:0005747
GO:0005758
GO:0005759
GO:0006091
GO:0006120
GO:0007005
GO:0008137
GO:0008637
GO:0009055
GO:0009060
GO:0012501
GO:0016020
GO:0016491
GO:0016651
GO:0032981
GO:0042773
GO:0042776
GO:0043226
GO:0045333
GO:0046034
GO:0046872
GO:0051536
GO:0051537
GO:0051539
GO:0051881
GO:0055086
GO:0065003
GO:0070469
GO:0072593
GO:1901135
HMNXSV003044879
HMNXSV003053191
Hs.109854.0.A1_3p_at
Hs.194079.0.A1_3p_at
Hs.240833.0.A1_3p_at
Hs.72063.0.A1_3p_at
ILMN_1728810
PH_hs_0025123
TC02002695.hg
TC02002696.hg
TC02002697.hg
TC02004793.hg
TC02004794.hg
X61100_rna1_at
g4826855_3p_a_at
p26102
Participates
as a member of
Substrate of LONP1 (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
NDUF subunits [mitochondrial matrix] (Homo sapiens)
as a component of
NDUFS1:2x4Fe-4S [mitochondrial matrix] (Homo sapiens)
Inferred To
NDUFS1 [mitochondrial matrix]
(Bos taurus)
NDUFS1 [mitochondrial matrix]
(Canis familiaris)
NDUFS1 [mitochondrial matrix]
(Dictyostelium discoideum)
NDUFS1 [mitochondrial matrix]
(Drosophila melanogaster)
NDUFS1 [mitochondrial matrix]
(Gallus gallus)
NDUFS1 [mitochondrial matrix]
(Mus musculus)
NDUFS1 [mitochondrial matrix]
(Rattus norvegicus)
NDUFS1 [mitochondrial matrix]
(Sus scrofa)
Ghost homologue of NDUFS1 [mitochondrial matrix]
(Caenorhabditis elegans)
Ghost homologue of NDUFS1 [mitochondrial matrix]
(Danio rerio)
Ghost homologue of NDUFS1 [mitochondrial matrix]
(Xenopus tropicalis)
Cross References
RefSeq
NP_001186910.1
,
NP_001186912.1
,
NP_004997.4
,
NP_001186913.1
,
NP_001186911.1
OpenTargets
ENSG00000023228
IntEnz
7.1.1.2
HPA
ENSG00000023228-NDUFS1
GeneCards
P28331
Ensembl
ENST00000423725
,
ENST00000432169
,
ENST00000635748
,
ENSG00000023228
,
ENSG00000283447
,
ENST00000637631
,
ENSP00000490583
,
ENST00000449699
,
ENSP00000399912
,
ENSP00000233190
,
ENST00000233190
,
ENSP00000392709
,
ENSP00000490898
,
ENSP00000489640
,
ENST00000440274
,
ENSP00000490092
,
ENSP00000409689
,
ENSP00000409766
,
ENST00000637733
,
ENSP00000489705
,
ENST00000637298
,
ENST00000637990
,
ENST00000455934
,
ENST00000636505
,
ENSP00000397760
,
ENSP00000490766
PRO
P28331
Pharos - Targets
P28331
Orphanet
16526
HMDB Protein
HMDBP00162
PDB
5XTH
,
5XTI
,
5XTB
,
5XTD
Interactors (5)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:O75489 NDUFS3
2
NDUFS3 [mitochondrial matrix]
(R-HSA-163927)
NDUFS3 [cytosol]
(R-HSA-9714751)
0.643
6
UniProt:O75380 NDUFS6
1
NDUFS6 [mitochondrial inner membrane]
(R-HSA-163922)
0.527
2
UniProt:Q99650 OSMR
1
OSMR [plasma membrane]
(R-HSA-448654)
0.499
4
UniProt:P13569 CFTR
17
PolyUb-CFTR [clathrin-coated endocytic vesicle membrane]
(R-HSA-8869104)
PolyUb-CFTR [plasma membrane]
(R-HSA-8867595)
CFTR [lysosomal membrane]
(R-HSA-5627279)
CFTR [Golgi-associated vesicle membrane]
(R-HSA-5627079)
CFTR [endosome membrane]
(R-HSA-6782965)
PolyUb-CFTR [endosome membrane]
(R-HSA-6782976)
CFTR F508del [endoplasmic reticulum membrane]
(R-HSA-8866834)
ub-CFTR F508del [cytosol]
(R-HSA-8866838)
ub-CFTR F508del [endoplasmic reticulum membrane]
(R-HSA-8866842)
CFTR G542* [plasma membrane]
(R-HSA-5678986)
CFTR G551D [plasma membrane]
(R-HSA-5678977)
CFTR W1282* [plasma membrane]
(R-HSA-5678976)
CFTR F508del [plasma membrane]
(R-HSA-5678978)
CFTR N1303K [plasma membrane]
(R-HSA-5678983)
CFTR [plasma membrane]
(R-HSA-383188)
CFTR [endoplasmic reticulum membrane]
(R-HSA-8866837)
misfolded CFTR [endoplasmic reticulum membrane]
(R-HSA-8868730)
0.483
7
UniProt:Q8IWL3 HSCB
5
HSCB [mitochondrial matrix]
(R-HSA-1299438)
HSCB [mitochondrial inner membrane]
(R-HSA-1299447)
HSCB(1-235) [mitochondrial inner membrane]
(R-HSA-1299443)
HSCB(1-30) [mitochondrial matrix]
(R-HSA-8986120)
HSCB(1-235) [mitochondrial intermembrane space]
(R-HSA-1299456)
0.462
6
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