Reactome | NDUFA9 [mitochondrial matrix]

NDUFA9 [mitochondrial matrix]

Stable Identifier

R-HSA-163942

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

NADH-ubiquinone oxidoreductase 39 kDa subunit

Locations in the PathwayBrowser

Expand all

Metabolism (Homo sapiens)

- Aerobic respiration and respiratory electron transport (Homo sapiens)
  - Respiratory electron transport (Homo sapiens)
    - Complex I biogenesis (Homo sapiens)
      - IP subcomplex binds NDUFAF3, NDUFAF4, TIMMDC1 to form Intermediate 1 (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
      - Intermediate 1 binds HP subcomplex to form Intermediate 2 (Homo sapiens)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
      - NDUF subunits bind to form the IP subcomplex (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
        
        550kDa complex [mitochondrial inner membrane] (Homo sapiens)
        
        315kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [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)
        
        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)
        
        Intermediate 1 [mitochondrial inner membrane] (Homo sapiens)
        
        IP subcomplex [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (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)
        
        Intermediate 1 (-NDUFAF3,4,7, TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)
    - Complex I oxidises NADH to NAD+, reduces CoQ to CoQH2 (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)
        
        Intermediate 1 (-NDUFAF3,4,7, TIMMDC1) [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)
        
        NDUFA9 [mitochondrial matrix] (Homo sapiens)

External Reference Information

External Reference

UniProt:Q16795 NDUFA9

Gene Names

NDUFA9, NDUFS2L

Chain

transit peptide:1-35, chain:36-377

Reference Genes

BioGPS Gene:4704 NDUFA9

COSMIC (genes):NDUFA9 NDUFA9

CTD Gene:4704 NDUFA9

dbSNP Gene:4704 NDUFA9

ENSEMBL:ENSG00000139180 NDUFA9

HGNC:7693 NDUFA9

KEGG:hsa:4704 NDUFA9

Monarch:4704 NDUFA9

NCBI Gene:4704 NDUFA9

OMIM:603834 NDUFA9

UCSC:Q16795 NDUFA9

Reference Transcript

RefSeq:NM_005002.5 NDUFA9

Other Identifiers

11757029_a_at

16747077

208969_PM_at

208969_at

242620_PM_at

242620_at

3401879

3401880

3401881

3401882

3401883

3401884

3401885

3401886

3401887

3401888

3401889

3401891

3401892

3401894

3401895

3401897

3401898

3401899

3401900

3401901

3401902

3401903

3401906

3401908

3401910

3401912

3401913

3401915

3401917

36205_at

3959298

3959304

3959305

4704

51307_at

72458_at

73284_at

7953243

86078_at

A_19_P00322045

A_19_P00810426

A_23_P76499

A_24_P361006

GE532274

GE61359

GE822567

GO:0003954

GO:0005515

GO:0005634

GO:0005739

GO:0005743

GO:0005759

GO:0006091

GO:0006120

GO:0006744

GO:0006814

GO:0007623

GO:0008137

GO:0009060

GO:0016491

GO:0031966

GO:0042776

GO:0044877

GO:0045271

GO:0055085

GO:0055086

GO:1901135

GO:1902600

HMNXSV003016368

Hs.269074.0.A1_3p_at

ILMN_1760741

L04490_at

PH_hs_0004290

PH_hs_0010136

TC12003205.hg

g5326822_3p_at

Participates

as a component of

NDUFA9:FAD [mitochondrial matrix] (Homo sapiens)

Inferred To

ND-39 [mitochondrial matrix] (Drosophila melanogaster)

NDUFA9 [mitochondrial matrix] (Bos taurus)

NDUFA9 [mitochondrial matrix] (Canis familiaris)

NDUFA9 [mitochondrial matrix] (Gallus gallus)

NDUFA9 [mitochondrial matrix] (Sus scrofa)

ndufa9 [mitochondrial matrix] (Dictyostelium discoideum)

ndufa9 [mitochondrial matrix] (Xenopus tropicalis)

nduf-9 [mitochondrial matrix] (Caenorhabditis elegans)

ndufa9a [mitochondrial matrix] (Danio rerio)

Ndufa9 [mitochondrial matrix] (Mus musculus)

Ndufa9 [mitochondrial matrix] (Rattus norvegicus)

Cross References

RefSeq

NP_004993.1

ENSEMBL

ENSG00000139180, ENSP00000266544, ENST00000266544

OpenTargets

ENSG00000139180

GeneCards

NDUFA9

HPA

ENSG00000139180-NDUFA9

PRO

Q16795

Pharos - Targets

Q16795

GlyGen

Q16795

HMDB Protein

HMDBP00177

PDB

5XTD, 5XTI, 5XTH, 5XTB

Interactors (15)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:O75489 NDUFS3	2	NDUFS3 [mitochondrial matrix] (R-HSA-163927) NDUFS3 [cytosol] (R-HSA-9714751)	0.788	10
UniProt:P19404 NDUFV2	1	NDUFV2 [mitochondrial inner membrane] (R-HSA-163939)	0.744	6
UniProt:O75380 NDUFS6	1	NDUFS6 [mitochondrial inner membrane] (R-HSA-163922)	0.725	7
UniProt:P42858 HTT	1	HTT [nucleoplasm] (R-HSA-9023590)	0.666	7
UniProt:P49821 NDUFV1	1	NDUFV1 [mitochondrial inner membrane] (R-HSA-163926)	0.643	4
UniProt:P03915 MT-ND5	1	MT-ND5 [mitochondrial inner membrane] (R-HSA-60565)	0.643	4
UniProt:Q9UI09 NDUFA12	1	NDUFA12 [mitochondrial inner membrane] (R-HSA-59864)	0.643	5
UniProt:O43181 NDUFS4	1	NDUFS4 [mitochondrial inner membrane] (R-HSA-163946)	0.643	5
UniProt:Q9P0J0 NDUFA13	8	NDUFA13 [mitochondrial inner membrane] (R-HSA-59936) NDUFA13(41-49) [mitochondrial inner membrane] (R-HSA-9839958) NDUFA13(100-108) [mitochondrial inner membrane] (R-HSA-9839959) NDUFA13(109-144) [mitochondrial inner membrane] (R-HSA-9839968) NDUFA13(2-19) [mitochondrial inner membrane] (R-HSA-9839966) NDUFA13(69-99) [mitochondrial inner membrane] (R-HSA-9839964) NDUFA13(20-40) [mitochondrial inner membrane] (R-HSA-9839963) NDUFA13(50-68) [mitochondrial inner membrane] (R-HSA-9839961)	0.643	3
UniProt:P56181 NDUFV3	1	NDUFV3 [mitochondrial inner membrane] (R-HSA-163943)	0.643	5
UniProt:P10809 HSPD1	6	HSPD1 [mitochondrial inner membrane] (R-HSA-1299455) HSPD1 [mitochondrial matrix] (R-HSA-1299448) HSPD1(1-573) [mitochondrial inner membrane] (R-HSA-1299442) HSPD1(1-26) [mitochondrial matrix] (R-HSA-8986171) HSPD1(1-573) [mitochondrial intermembrane space] (R-HSA-1267963) HSPD1(1-573) [cytosol] (R-HSA-1267989)	0.615	4
UniProt:O95139 NDUFB6	1	NDUFB6 [mitochondrial inner membrane] (R-HSA-59938)	0.527	3
UniProt:O14561 NDUFAB1	3	NDUFAB1 [mitochondrial inner membrane] (R-HSA-163925) octanoyl-S112-NDUFAB1 [mitochondrial inner membrane] (R-HSA-9857608) Mal-PPANT-S112-NDUFAB1 [mitochondrial inner membrane] (R-HSA-8934586)	0.527	2
UniProt:O43920 NDUFS5	1	NDUFS5 [mitochondrial inner membrane] (R-HSA-60226)	0.527	2
UniProt:P78537 BLOC1S1	2	BLOC1S1 [Golgi membrane] (R-HSA-434324) BLOC1S1 [lysosomal membrane] (R-HSA-429821)	0.524	3

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