Reactome | NDUFA1 [mitochondrial inner membrane]

NDUFA1 [mitochondrial inner membrane]

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)

- Aerobic respiration 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

ENSEMBL_homo_sapiens_GENE:ENSG00000125356.7 NDUFA1

HGNC:7683 NDUFA1

KEGG Gene (Homo sapiens):4694 NDUFA1

Monarch:4694 NDUFA1

NCBI Gene:4694 NDUFA1

OMIM:300078 NDUFA1

Reference Transcript

RefSeq:NM_004541.3 NDUFA1

Other Identifiers

11723291_a_at

11753867_a_at

11756125_x_at

17106604

202298_PM_at

202298_at

36169_at

3988989

3988990

3988991

3988994

4694

8169659

A_23_P159833

A_24_P348649

GE61801

GO:0003824

GO:0005215

GO:0005739

GO:0005743

GO:0005747

GO:0006091

GO:0006120

GO:0007005

GO:0008137

GO:0009060

GO:0016020

GO:0016491

GO:0031966

GO:0032981

GO:0042776

GO:0043226

GO:0055086

GO:0065003

GO:0070469

GO:1901135

HMNXSV003032683

ILMN_1784286

PH_hs_0035451

TC0X000589.hg

g13699820_3p_at

Participates

as a component of

HP subcomplex [mitochondrial inner membrane] (Homo sapiens)

Inferred To

LOC481033 [mitochondrial inner membrane] (Canis familiaris)

ND-MWFE [mitochondrial inner membrane] (Drosophila melanogaster)

Ghost homologue of NDUFA1 [mitochondrial inner membrane] (Caenorhabditis elegans)

Ghost homologue of NDUFA1 [mitochondrial inner membrane] (Rattus norvegicus)

Ghost homologue of NDUFA1 [mitochondrial inner membrane] (Xenopus tropicalis)

NDUFA1 [mitochondrial inner membrane] (Bos taurus)

NDUFA1 [mitochondrial inner membrane] (Gallus gallus)

NDUFA1 [mitochondrial inner membrane] (Sus scrofa)

Ndufa1 [mitochondrial inner membrane] (Mus musculus)

zgc:86674 [mitochondrial inner membrane] (Danio rerio)

Cross References

RefSeq

NP_004532.1

OpenTargets

ENSG00000125356

GeneCards

O15239

HPA

ENSG00000125356-NDUFA1

Ensembl

ENSG00000125356, ENSP00000360492, ENST00000371437

PRO

O15239

Pharos - Targets

O15239

Orphanet

16872

PDB

5XTD, 5XTI, 5XTH, 5XTC

HMDB Protein

HMDBP00127

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