Reactome | NDUFA1 [mitochondrial inner membrane]

Reactome will be unavailable due to host maintenance from Friday, April 27 at 6pm to Sunday, April 29 at 11:55pm.

During the outage, you may wish to use our mirror site at reactome.ncpsb.org.

NDUFA1

Stable Identifier

R-HSA-60212

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

Synonyms

NADH-ubiquinone oxidoreductase MWFE subunit , Complex I-MWFE, CI-MWFE

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)
        
        Intermediate 1 binds HP subcomplex to form Intermediate 2 (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 binds MT-ND1:NDUFAF5:NDUFAF6 to form a 315kDa subcomplex (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        ND4, ND5 bind the 550kDa complex to form the 815kDa complex (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        815kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        Peripheral arm subunits bind the 815kDa complex to form a 980kDa complex (Homo sapiens)
        
        815kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        980kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        815kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        The 315kDa subcomplex binds the 370kDa subcomplex to form the 550kDa complex (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (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)
        
        815kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
        
        Complex I [mitochondrial inner membrane] (Homo sapiens)
        
        815kDa complex (-MCIA, NDUFAF3,4,5,6,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex (-MCIA, NDUFAF3,4,5,6,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex (-NDUFAF3,4,5,6,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 (-NDUFAF3,4,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)
      - Complex I oxidises NADH to NAD+, reduces CoQ to QH2 (Homo sapiens)
        
        Complex I [mitochondrial inner membrane] (Homo sapiens)
        
        815kDa complex (-MCIA, NDUFAF3,4,5,6,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        550kDa complex (-MCIA, NDUFAF3,4,5,6,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex (-NDUFAF3,4,5,6,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 (-NDUFAF3,4,7,TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA1 [mitochondrial inner membrane] (Homo sapiens)

External Reference Information

External Reference

UniProt:O15239 NDUFA1

Gene Names

NDUFA1

Chain

chain:1-70

Reference Genes

BioGPS Gene:4694 NDUFA1

COSMIC (genes):NDUFA1 NDUFA1

CTD Gene:4694 NDUFA1

dbSNP Gene:4694 NDUFA1

ENSEMBL:ENSG00000125356 NDUFA1

HGNC:7683 NDUFA1

KEGG Gene (Homo sapiens):hsa:4694 NDUFA1

Monarch:4694 NDUFA1

NCBI Gene:4694 NDUFA1

OMIM:300078 NDUFA1

Reference Transcript

RefSeq:NM_004541.3 NDUFA1

Other Identifiers

0004060010

11723291_a_at

11753867_a_at

11756125_x_at

17106604

202298_at

29768

36169_at

4694

54359

8169659

A_23_P159833

A_24_P348649

AAD00084

AAH00266

AB451304

AB451435

BAG70118

BAG70249

BC000266

CAA57489

CAG33170

CCDS14590

CH471161

CR456889

EAW89840

ENSG00000125356

ENSP00000360492

ENST00000371437

EntrezGene:4694

g13699820_3p_at

GE61801

GO:0003674

GO:0005575

GO:0005622

GO:0005623

GO:0005737

GO:0005739

GO:0005743

GO:0005747

GO:0005829

GO:0006091

GO:0006120

GO:0006461

GO:0007005

GO:0008137

GO:0008150

GO:0016020

GO:0016021

GO:0016491

GO:0022607

GO:0031966

GO:0032981

GO:0034641

GO:0043226

GO:0043234

GO:0044281

GO:0055114

GO:0065003

GO:0070469

HGNC:7683

HPA029768

HPA054359

ILMN_1784286

IPR017384

MIM:252010

MIM:300078

NDUFA1

NDUFA1-201

NM_004541

NP_004532

PF15879

PH_hs_0035451

Q6IBB5

TC0X000589.hg

U54993

uc004esc.5

UPI000013018C

X81900

Participant Of

hasComponent

HP subcomplex [mitochondrial inner membrane]

Inferred To

Homologues of NDUFA1 [mitochondrial inner membrane] (Rattus norvegicus)

Homologues of NDUFA1 [mitochondrial inner membrane] (Sus scrofa)

Ghost homologue of NDUFA1 [mitochondrial inner membrane] (Canis familiaris)

Ghost homologue of NDUFA1 [mitochondrial inner membrane] (Drosophila melanogaster)

NDUFA1 [mitochondrial inner membrane] (Bos taurus)

NDUFA1 [mitochondrial inner membrane] (Danio rerio)

NDUFA1 [mitochondrial inner membrane] (Gallus gallus)

NDUFA1 [mitochondrial inner membrane] (Mus musculus)

NDUFA1 [mitochondrial inner membrane] (Rattus norvegicus)

NDUFA1 [mitochondrial inner membrane] (Sus scrofa)

NDUFA1 [mitochondrial inner membrane] (Taeniopygia guttata)

NDUFA1 [mitochondrial inner membrane] (Xenopus tropicalis)

Cross References

RefSeq

NP_004532.1

GeneCards

O15239

PRO

O15239

Orphanet

16872

PDB

5XTI, 5XTC, 5XTD, 5XTH

HMDB Protein

HMDBP00127