Reactome | NDUFA5 [mitochondrial inner membrane]

NDUFA5 [mitochondrial inner membrane]

Stable Identifier

R-HSA-60599

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

Synonyms

NADH-ubiquinone oxidoreductase 13 kDa-B subunit , Complex I-13Kd-B, CI-13Kd-B, Complex I subunit B13

Locations in the PathwayBrowser

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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)
        
        NDUFA5 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA5 [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)
        
        NDUFA5 [mitochondrial inner membrane] (Homo sapiens)
        
        Intermediate 2 [mitochondrial inner membrane] (Homo sapiens)
        
        HP subcomplex [mitochondrial inner membrane] (Homo sapiens)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [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)
        
        NDUFA5 [mitochondrial inner membrane] (Homo sapiens)

External Reference Information

External Reference

UniProt:Q16718 NDUFA5

Gene Names

NDUFA5

Chain

initiator methionine:1, chain:2-116

Reference Genes

BioGPS Gene:4698 NDUFA5

COSMIC (genes):NDUFA5 NDUFA5

CTD Gene:4698 NDUFA5

dbSNP Gene:4698 NDUFA5

ENSEMBL:ENSG00000128609 NDUFA5

ENSEMBL:ENSG00000128609.16 NDUFA5

HGNC:7688 NDUFA5

KEGG Gene (Homo sapiens):4698 NDUFA5

Monarch:4698 NDUFA5

NCBI Gene:4698 NDUFA5

OMIM:601677 NDUFA5

UCSC:Q16718 NDUFA5

Reference Transcript

Other Identifiers

11754079_s_at

201304_PM_at

201304_at

2523421

2523422

2523423

2523424

3070644

3070645

3070646

3070647

3070648

3070649

3070650

3070655

3070657

3070659

3070660

3070661

3070662

3070663

3070664

3070666

3070669

3070672

3070674

3070675

3070676

3096901

3690326

38462_at

4698

74839_r_at

8142663

A_23_P215751

A_32_P96641

GE58352

GO:0003824

GO:0005215

GO:0005515

GO:0005739

GO:0005743

GO:0005747

GO:0006091

GO:0006120

GO:0007005

GO:0008137

GO:0009060

GO:0016020

GO:0016491

GO:0022904

GO:0032981

GO:0042776

GO:0043226

GO:0045271

GO:0055086

GO:0065003

GO:0070469

GO:1901135

HMNXSV003035421

ILMN_1759973

ILMN_1764095

ILMN_1824709

PH_hs_0027720

PH_hs_0031370

TC07001821.hg

U53468_at

g13699821_3p_at

Participates

as a component of

HP subcomplex [mitochondrial inner membrane] (Homo sapiens)

Other forms of this molecule

NDUFA5 [endoplasmic reticulum membrane]

Inferred To

NDUFA5 [mitochondrial inner membrane] (Bos taurus)

NDUFA5 [mitochondrial inner membrane] (Caenorhabditis elegans)

NDUFA5 [mitochondrial inner membrane] (Canis familiaris)

NDUFA5 [mitochondrial inner membrane] (Gallus gallus)

NDUFA5 [mitochondrial inner membrane] (Sus scrofa)

Ndufa5 [mitochondrial inner membrane] (Mus musculus)

Ndufa5 [mitochondrial inner membrane] (Rattus norvegicus)

ND-13B [mitochondrial inner membrane] (Drosophila melanogaster)

zgc:114172 [mitochondrial inner membrane] (Danio rerio)

ndufa5 [mitochondrial inner membrane] (Xenopus tropicalis)

Cross References

RefSeq

NP_001269351.1, NP_001269348.1, NP_004991.1, NP_001269350.1, NP_001269349.1

OpenTargets

ENSG00000128609

HPA

ENSG00000128609-NDUFA5

GeneCards

Q16718

Ensembl

ENSG00000128609, ENSP00000347988, ENSP00000417142, ENST00000471770, ENST00000355749

PRO

Q16718

Pharos - Targets

Q16718

Orphanet

24738

PDB

5XTD, 5XTB, 5XTH, 5XTI

HMDB Protein

HMDBP00175

Interactors (3)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:O75489 NDUFS3	2	NDUFS3 [mitochondrial matrix] (R-HSA-163927) NDUFS3 [cytosol] (R-HSA-9714751)	0.8	12
UniProt:Q92993 KAT5	1	KAT5 [nucleoplasm] (R-HSA-3321979)	0.556	3
UniProt:Q08379 GOLGA2	3	p-S37-GOLGA2 [Golgi membrane] (R-HSA-2172179) GOLGA2 [endoplasmic reticulum-Golgi intermediate compartment membrane] (R-HSA-2167933) GOLGA2 [Golgi membrane] (R-HSA-6808764)	0.488	2

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