UniProt Accession

 NDUS8_HUMAN; O00217; B2RB86; Q0VDA8 

Genbank Protein ID

 U65579; AF038406; AK314546; BC119754 

Genbank Nucleotide ID

 AAB51776.1; AAC34273.1; BAG37133.1; AAI19755.1 

Protein Name

 NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 

Protein Synonyms/Alias

 Complex I-23kD; CI-23kD; NADH-ubiquinone oxidoreductase 23 kDa subunit; TYKY subunit 

Gene Name

 NDUFS8 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
49	QDPEMDMKSVTDRAA	ubiquitination	[1, 2]
88	TINYPFEKGPLSPRF	ubiquitination	[1, 2, 3]

Reference

 [1] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [2] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661]
 [3] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 

Functional Description

 Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). May donate electrons to ubiquinone. 

Sequence Annotation

 DOMAIN 102 131 4Fe-4S ferredoxin-type 1.
 DOMAIN 141 170 4Fe-4S ferredoxin-type 2.
 METAL 111 111 Iron-sulfur 1 (4Fe-4S) (By similarity).
 METAL 114 114 Iron-sulfur 1 (4Fe-4S) (By similarity).
 METAL 117 117 Iron-sulfur 1 (4Fe-4S) (By similarity).
 METAL 121 121 Iron-sulfur 2 (4Fe-4S) (By similarity).
 METAL 150 150 Iron-sulfur 2 (4Fe-4S) (By similarity).
 METAL 153 153 Iron-sulfur 2 (4Fe-4S) (By similarity).
 METAL 156 156 Iron-sulfur 2 (4Fe-4S) (By similarity).
 METAL 160 160 Iron-sulfur 1 (4Fe-4S) (By similarity).  

Keyword

 4Fe-4S; Complete proteome; Disease mutation; Electron transport; Iron; Iron-sulfur; Leigh syndrome; Metal-binding; Mitochondrion; NAD; Oxidoreductase; Reference proteome; Repeat; Respiratory chain; Transit peptide; Transport; Ubiquinone. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 210 AA 

Protein Sequence

Gene Ontology

 GO:0005747; C:mitochondrial respiratory chain complex I; IDA:UniProtKB.
 GO:0051539; F:4 iron, 4 sulfur cluster binding; IEA:UniProtKB-KW.
 GO:0009055; F:electron carrier activity; IEA:InterPro.
 GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
 GO:0008137; F:NADH dehydrogenase (ubiquinone) activity; NAS:UniProtKB.
 GO:0006120; P:mitochondrial electron transport, NADH to ubiquinone; NAS:UniProtKB.
 GO:0032981; P:mitochondrial respiratory chain complex I assembly; IMP:UniProtKB.
 GO:0006979; P:response to oxidative stress; IDA:UniProtKB.
 GO:0044281; P:small molecule metabolic process; TAS:Reactome. 

Interpro

 IPR001450; 4Fe4S-bd_dom.
 IPR017896; 4Fe4S_Fe-S-bd.
 IPR017900; 4Fe4S_Fe_S_CS.
 IPR012285; Fum_reductase_C.
 IPR010226; NADH_quinone_OxRdtase_chainI. 

Pfam

 PF12838; Fer4_7 

SMART

  

PROSITE

 PS00198; 4FE4S_FER_1
 PS51379; 4FE4S_FER_2 

PRINTS