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Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11

Gene

NDUFA11

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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 (By similarity).By similarity

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11
Alternative name(s):
Complex I-B14.7
Short name:
CI-B14.7
NADH-ubiquinone oxidoreductase subunit B14.7
Gene namesi
Name:NDUFA11
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:20371. NDUFA11.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei21 – 4323HelicalSequence analysisAdd
BLAST
Transmembranei58 – 8023HelicalSequence analysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex I deficiency (MT-C1D)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
See also OMIM:252010

Organism-specific databases

MalaCardsiNDUFA11.
MIMi252010. phenotype.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA134914606.

Chemistry

ChEMBLiCHEMBL2363065.

Polymorphism and mutation databases

BioMutaiNDUFA11.
DMDMi52000823.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methionineiRemovedBy similarity1 Publication
Chaini2 – 141140NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11PRO_0000118841Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylalanineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ86Y39.
MaxQBiQ86Y39.
PaxDbiQ86Y39.
PeptideAtlasiQ86Y39.
PRIDEiQ86Y39.
TopDownProteomicsiQ86Y39-1. [Q86Y39-1]

PTM databases

iPTMnetiQ86Y39.
PhosphoSiteiQ86Y39.

Expressioni

Gene expression databases

BgeeiENSG00000174886.
CleanExiHS_NDUFA11.
ExpressionAtlasiQ86Y39. baseline and differential.
GenevisibleiQ86Y39. HS.

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits.1 Publication

Protein-protein interaction databases

BioGridi125981. 40 interactions.
IntActiQ86Y39. 39 interactions.
STRINGi9606.ENSP00000389160.

Structurei

3D structure databases

ProteinModelPortaliQ86Y39.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFA11 subunit family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiENOG410IYXZ. Eukaryota.
ENOG4111P1Q. LUCA.
GeneTreeiENSGT00390000012434.
HOGENOMiHOG000113751.
HOVERGENiHBG052568.
InParanoidiQ86Y39.
KOiK03956.
OMAiFLGARTH.
OrthoDBiEOG091G0SEB.
PhylomeDBiQ86Y39.
TreeFamiTF314729.

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q86Y39-1) [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
MAPKVFRQYW DIPDGTDCHR KAYSTTSIAS VAGLTAAAYR VTLNPPGTFL
60 70 80 90 100
EGVAKVGQYT FTAAAVGAVF GLTTCISAHV REKPDDPLNY FLGGCAGGLT
110 120 130 140
LGARTHNYGI GAAACVYFGI AASLVKMGRL EGWEVFAKPK V
Length:141
Mass (Da):14,852
Last modified:January 23, 2007 - v3
Checksum:i379D58482D7E8BDB
GO
Isoform 2 (identifier: Q86Y39-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     105-141: THNYGIGAAA...GWEVFAKPKV → KTGSHCVVQA...LNTLGSNPRT

Show »
Length:228
Mass (Da):23,411
Checksum:iB7DBF575887BD4D9
GO

Sequence cautioni

The sequence BAC87088 differs from that shown.Erroneous CDS prediction.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Isoform 2 (identifier: Q86Y39-2)
Sequence conflicti160 – 1601S → G in BAC87088 (PubMed:14702039).Curated
Sequence conflicti221 – 2211T → A in BAC87088 (PubMed:14702039).Curated

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei105 – 14137THNYG…AKPKV → KTGSHCVVQAGLKLLASSSP HTSASQSAGIIGMSHCVQRF WVPSSSACLEVLSGESTDVH ACSSTRGACNSSGSRPLPEL GARASGSLRKGGHTHPAPRG AGALTPVQALIESLLNTLGS NPRT in isoform 2. 1 PublicationVSP_033813Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ539081 mRNA. Translation: CAD62165.1.
AK127692 mRNA. Translation: BAC87088.1. Sequence problems.
AC024592 Genomic DNA. No translation available.
AC104532 Genomic DNA. No translation available.
BC069045 mRNA. Translation: AAH69045.1.
CCDSiCCDS12155.1. [Q86Y39-1]
CCDS54203.1. [Q86Y39-2]
RefSeqiNP_001180304.1. NM_001193375.1. [Q86Y39-2]
NP_783313.1. NM_175614.4. [Q86Y39-1]
UniGeneiHs.406062.

Genome annotation databases

EnsembliENST00000308961; ENSP00000311740; ENSG00000174886. [Q86Y39-1]
ENST00000418389; ENSP00000389160; ENSG00000174886. [Q86Y39-2]
GeneIDi126328.
KEGGihsa:126328.
UCSCiuc002mdp.3. human. [Q86Y39-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ539081 mRNA. Translation: CAD62165.1.
AK127692 mRNA. Translation: BAC87088.1. Sequence problems.
AC024592 Genomic DNA. No translation available.
AC104532 Genomic DNA. No translation available.
BC069045 mRNA. Translation: AAH69045.1.
CCDSiCCDS12155.1. [Q86Y39-1]
CCDS54203.1. [Q86Y39-2]
RefSeqiNP_001180304.1. NM_001193375.1. [Q86Y39-2]
NP_783313.1. NM_175614.4. [Q86Y39-1]
UniGeneiHs.406062.

3D structure databases

ProteinModelPortaliQ86Y39.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi125981. 40 interactions.
IntActiQ86Y39. 39 interactions.
STRINGi9606.ENSP00000389160.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

iPTMnetiQ86Y39.
PhosphoSiteiQ86Y39.

Polymorphism and mutation databases

BioMutaiNDUFA11.
DMDMi52000823.

Proteomic databases

EPDiQ86Y39.
MaxQBiQ86Y39.
PaxDbiQ86Y39.
PeptideAtlasiQ86Y39.
PRIDEiQ86Y39.
TopDownProteomicsiQ86Y39-1. [Q86Y39-1]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000308961; ENSP00000311740; ENSG00000174886. [Q86Y39-1]
ENST00000418389; ENSP00000389160; ENSG00000174886. [Q86Y39-2]
GeneIDi126328.
KEGGihsa:126328.
UCSCiuc002mdp.3. human. [Q86Y39-1]

Organism-specific databases

CTDi126328.
GeneCardsiNDUFA11.
HGNCiHGNC:20371. NDUFA11.
MalaCardsiNDUFA11.
MIMi252010. phenotype.
612638. gene.
neXtProtiNX_Q86Y39.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA134914606.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IYXZ. Eukaryota.
ENOG4111P1Q. LUCA.
GeneTreeiENSGT00390000012434.
HOGENOMiHOG000113751.
HOVERGENiHBG052568.
InParanoidiQ86Y39.
KOiK03956.
OMAiFLGARTH.
OrthoDBiEOG091G0SEB.
PhylomeDBiQ86Y39.
TreeFamiTF314729.

Enzyme and pathway databases

ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Miscellaneous databases

GenomeRNAii126328.
PROiQ86Y39.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000174886.
CleanExiHS_NDUFA11.
ExpressionAtlasiQ86Y39. baseline and differential.
GenevisibleiQ86Y39. HS.

Family and domain databases

ProtoNetiSearch...

Entry informationi

Entry nameiNDUAB_HUMAN
AccessioniPrimary (citable) accession number: Q86Y39
Secondary accession number(s): C9JT23, Q6ZS66
Entry historyi
Integrated into UniProtKB/Swiss-Prot: September 13, 2004
Last sequence update: January 23, 2007
Last modified: September 7, 2016
This is version 115 of the entry and version 3 of the sequence. [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

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.