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Protein

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial

Gene

NDUFB8

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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.1 Publication

GO - Molecular functioni

  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

  • mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  • mitochondrial respiratory chain complex I assembly Source: UniProtKB

Keywordsi

Biological processElectron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import
R-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial
Alternative name(s):
Complex I-ASHI
Short name:
CI-ASHI
NADH-ubiquinone oxidoreductase ASHI subunit
Gene namesi
Name:NDUFB8
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

EuPathDBiHostDB:ENSG00000166136.15
HGNCiHGNC:7703 NDUFB8
MIMi602140 gene
neXtProtiNX_O95169

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei133 – 153HelicalSequence analysisAdd BLAST21

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi4714
OpenTargetsiENSG00000166136
Orphaneti70474 Leigh syndrome with cardiomyopathy
PharmGKBiPA31514

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFB8

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 28MitochondrionBy similarityAdd BLAST28
ChainiPRO_000002004629 – 186NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrialAdd BLAST158

Proteomic databases

EPDiO95169
MaxQBiO95169
PaxDbiO95169
PeptideAtlasiO95169
PRIDEiO95169
ProteomicsDBi50683
TopDownProteomicsiO95169-1 [O95169-1]

PTM databases

iPTMnetiO95169
PhosphoSitePlusiO95169
SwissPalmiO95169

Expressioni

Gene expression databases

BgeeiENSG00000166136 Expressed in 238 organ(s), highest expression level in endothelial cell
CleanExiHS_NDUFB8
ExpressionAtlasiO95169 baseline and differential
GenevisibleiO95169 HS

Organism-specific databases

HPAiHPA003886

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits.2 Publications

Protein-protein interaction databases

BioGridi110794, 75 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiO95169
IntActiO95169, 27 interactors
MINTiO95169
STRINGi9606.ENSP00000299166

Structurei

3D structure databases

ProteinModelPortaliO95169
SMRiO95169
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFB8 subunit family.Curated

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG4040 Eukaryota
ENOG4111WEY LUCA
GeneTreeiENSGT00390000000628
HOGENOMiHOG000012999
HOVERGENiHBG000931
InParanoidiO95169
KOiK03964
OMAiKLCAANP
OrthoDBiEOG091G15KV
PhylomeDBiO95169
TreeFamiTF317319

Family and domain databases

InterProiView protein in InterPro
IPR008699 NDUFB8
IPR016551 Ndufb8_metazoa
PANTHERiPTHR12840 PTHR12840, 1 hit
PfamiView protein in Pfam
PF05821 NDUF_B8, 1 hit
PIRSFiPIRSF009288 NDUB8, 1 hit

Sequences (3+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: O95169-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MAVARAGVLG VQWLQRASRN VMPLGARTAS HMTKDMFPGP YPRTPEERAA
60 70 80 90 100
AAKKYNMRVE DYEPYPDDGM GYGDYPKLPD RSQHERDPWY SWDQPGLRLN
110 120 130 140 150
WGEPMHWHLD MYNRNRVDTS PTPVSWHVMC MQLFGFLAFM IFMCWVGDVY
160 170 180
PVYQPVGPKQ YPYNNLYLER GGDPSKEPER VVHYEI
Length:186
Mass (Da):21,766
Last modified:May 1, 1999 - v1
Checksum:i518459D44965D202
GO
Isoform 2 (identifier: O95169-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     157-172: GPKQYPYNNLYLERGG → CRHHFSYNLGFLSALG
     173-186: Missing.

Note: No experimental confirmation available.
Show »
Length:172
Mass (Da):20,039
Checksum:iD11BE3F6BEDD1658
GO
Isoform 3 (identifier: O95169-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-31: Missing.

Note: No experimental confirmation available. Derived from EST data.
Show »
Length:155
Mass (Da):18,479
Checksum:iB409F622289FC308
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E9PQ68E9PQ68_HUMAN
Uncharacterized protein
159Annotation score:
E9PIZ8E9PIZ8_HUMAN
NADH dehydrogenase [ubiquinone] 1 b...
NDUFB8
71Annotation score:
K9J7I2K9J7I2_HUMAN
Uncharacterized protein
107Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti44T → I in CAB45691 (PubMed:11230166).Curated1
Sequence conflicti44T → I in CAG38521 (Ref. 5) Curated1
Sequence conflicti171G → S in AAD27761 (PubMed:11042152).Curated1
Sequence conflicti184Y → N in CAB45691 (PubMed:11230166).Curated1
Sequence conflicti184Y → N in CAG38521 (Ref. 5) Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0548421 – 31Missing in isoform 3. 1 PublicationAdd BLAST31
Alternative sequenceiVSP_054843157 – 172GPKQY…LERGG → CRHHFSYNLGFLSALG in isoform 2. 1 PublicationAdd BLAST16
Alternative sequenceiVSP_054844173 – 186Missing in isoform 2. 1 PublicationAdd BLAST14

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044958 mRNA Translation: AAD05422.1
AF115968 mRNA Translation: AAP97239.1
AF077028 mRNA Translation: AAD27761.1
AL080056 mRNA Translation: CAB45691.1
CR533490 mRNA Translation: CAG38521.1
AK289579 mRNA Translation: BAF82268.1
AK295867 mRNA Translation: BAG58666.1
AL133352 Genomic DNA No translation available.
CH471066 Genomic DNA Translation: EAW49819.1
CH471066 Genomic DNA Translation: EAW49820.1
BC000466 mRNA Translation: AAH00466.1
BC019276 mRNA Translation: AAH19276.1
BI602529 mRNA No translation available.
Y18944 Genomic DNA Translation: CAB46274.1
CCDSiCCDS65916.1 [O95169-3]
CCDS65917.1 [O95169-2]
CCDS7497.1 [O95169-1]
PIRiJE0382
RefSeqiNP_001271296.1, NM_001284367.1 [O95169-2]
NP_001271297.1, NM_001284368.1 [O95169-3]
NP_004995.1, NM_005004.3 [O95169-1]
UniGeneiHs.523215

Genome annotation databases

EnsembliENST00000299166; ENSP00000299166; ENSG00000166136 [O95169-1]
ENST00000370320; ENSP00000359344; ENSG00000166136 [O95169-2]
ENST00000370322; ENSP00000359346; ENSG00000166136 [O95169-3]
GeneIDi4714
KEGGihsa:4714
UCSCiuc001kri.3 human [O95169-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF044958 mRNA Translation: AAD05422.1
AF115968 mRNA Translation: AAP97239.1
AF077028 mRNA Translation: AAD27761.1
AL080056 mRNA Translation: CAB45691.1
CR533490 mRNA Translation: CAG38521.1
AK289579 mRNA Translation: BAF82268.1
AK295867 mRNA Translation: BAG58666.1
AL133352 Genomic DNA No translation available.
CH471066 Genomic DNA Translation: EAW49819.1
CH471066 Genomic DNA Translation: EAW49820.1
BC000466 mRNA Translation: AAH00466.1
BC019276 mRNA Translation: AAH19276.1
BI602529 mRNA No translation available.
Y18944 Genomic DNA Translation: CAB46274.1
CCDSiCCDS65916.1 [O95169-3]
CCDS65917.1 [O95169-2]
CCDS7497.1 [O95169-1]
PIRiJE0382
RefSeqiNP_001271296.1, NM_001284367.1 [O95169-2]
NP_001271297.1, NM_001284368.1 [O95169-3]
NP_004995.1, NM_005004.3 [O95169-1]
UniGeneiHs.523215

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTCelectron microscopy3.70c34-186[»]
5XTDelectron microscopy3.70c34-186[»]
5XTHelectron microscopy3.90c34-186[»]
5XTIelectron microscopy17.40Bc/c34-186[»]
ProteinModelPortaliO95169
SMRiO95169
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110794, 75 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiO95169
IntActiO95169, 27 interactors
MINTiO95169
STRINGi9606.ENSP00000299166

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

PTM databases

iPTMnetiO95169
PhosphoSitePlusiO95169
SwissPalmiO95169

Polymorphism and mutation databases

BioMutaiNDUFB8

Proteomic databases

EPDiO95169
MaxQBiO95169
PaxDbiO95169
PeptideAtlasiO95169
PRIDEiO95169
ProteomicsDBi50683
TopDownProteomicsiO95169-1 [O95169-1]

Protocols and materials databases

DNASUi4714
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000299166; ENSP00000299166; ENSG00000166136 [O95169-1]
ENST00000370320; ENSP00000359344; ENSG00000166136 [O95169-2]
ENST00000370322; ENSP00000359346; ENSG00000166136 [O95169-3]
GeneIDi4714
KEGGihsa:4714
UCSCiuc001kri.3 human [O95169-1]

Organism-specific databases

CTDi4714
DisGeNETi4714
EuPathDBiHostDB:ENSG00000166136.15
GeneCardsiNDUFB8
HGNCiHGNC:7703 NDUFB8
HPAiHPA003886
MIMi602140 gene
neXtProtiNX_O95169
OpenTargetsiENSG00000166136
Orphaneti70474 Leigh syndrome with cardiomyopathy
PharmGKBiPA31514
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4040 Eukaryota
ENOG4111WEY LUCA
GeneTreeiENSGT00390000000628
HOGENOMiHOG000012999
HOVERGENiHBG000931
InParanoidiO95169
KOiK03964
OMAiKLCAANP
OrthoDBiEOG091G15KV
PhylomeDBiO95169
TreeFamiTF317319

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import
R-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis

Miscellaneous databases

GeneWikiiNDUFB8
GenomeRNAii4714
PROiPR:O95169
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000166136 Expressed in 238 organ(s), highest expression level in endothelial cell
CleanExiHS_NDUFB8
ExpressionAtlasiO95169 baseline and differential
GenevisibleiO95169 HS

Family and domain databases

InterProiView protein in InterPro
IPR008699 NDUFB8
IPR016551 Ndufb8_metazoa
PANTHERiPTHR12840 PTHR12840, 1 hit
PfamiView protein in Pfam
PF05821 NDUF_B8, 1 hit
PIRSFiPIRSF009288 NDUB8, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiNDUB8_HUMAN
AccessioniPrimary (citable) accession number: O95169
Secondary accession number(s): A8K0L4
, Q5W143, Q5W144, Q5W145, Q9UG53, Q9UJR4, Q9UQF3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: May 1, 1999
Last modified: November 7, 2018
This is version 170 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
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