Q91WD5 · NDUS2_MOUSE

  • Protein
    NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
  • Gene
    Ndufs2
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (PubMed:26437605, PubMed:29887397, PubMed:31297047, PubMed:38575788).
Essential for the catalytic activity and assembly of complex I (PubMed:26437605, PubMed:29887397, PubMed:31297047).
Redox-sensitive, critical component of the oxygen-sensing pathway in the pulmonary vasculature which plays a key role in acute pulmonary oxygen-sensing and hypoxic pulmonary vasoconstriction (PubMed:30922174).
Plays an important role in carotid body sensing of hypoxia (PubMed:26437605, PubMed:29887397).
Essential for glia-like neural stem and progenitor cell proliferation, differentiation and subsequent oligodendrocyte or neuronal maturation (PubMed:31297047).

Catalytic activity

Cofactor

[4Fe-4S] cluster (UniProtKB | Rhea| CHEBI:49883 )
Note: Binds 1 [4Fe-4S] cluster.

Features

Showing features for binding site.

TypeIDPosition(s)Description
Binding site326[4Fe-4S] cluster (UniProtKB | ChEBI)
Binding site332[4Fe-4S] cluster (UniProtKB | ChEBI)
Binding site347[4Fe-4S] cluster (UniProtKB | ChEBI)

GO annotations

AspectTerm
Cellular Componentmitochondrial inner membrane
Cellular Componentmitochondrion
Cellular Componentnucleoplasm
Cellular Componentrespiratory chain complex I
Molecular Function4 iron, 4 sulfur cluster binding
Molecular Functionmetal ion binding
Molecular FunctionNAD binding
Molecular FunctionNADH dehydrogenase (ubiquinone) activity
Molecular FunctionNADH dehydrogenase activity
Molecular Functionoxygen sensor activity
Molecular Functionquinone binding
Molecular Functionubiquitin protein ligase binding
Biological Processaerobic respiration
Biological Processcellular response to oxygen levels
Biological Processgliogenesis
Biological Processmitochondrial electron transport, NADH to ubiquinone
Biological Processmitochondrial respiratory chain complex I assembly
Biological Processneural precursor cell proliferation
Biological Processneurogenesis
Biological Processproton motive force-driven mitochondrial ATP synthesis

Keywords

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
  • EC number
  • Alternative names
    • Complex I-49kD (CI-49kD)
    • NADH-ubiquinone oxidoreductase 49 kDa subunit

Gene names

    • Name
      Ndufs2

Organism names

  • Taxonomic identifier
  • Strains
    • BALB/cJ
    • C57BL/6J
    • FVB/N
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus

Accessions

  • Primary accession
    Q91WD5
  • Secondary accessions
    • Q3TNA8
    • Q3U5Y9
    • Q3UJX7
    • Q99L23

Proteomes

Organism-specific databases

Subcellular Location

Phenotypes & Variants

Disruption phenotype

Knockout mice show early perinatal death and major defects in the CNS, compromising especially the postnatal development of dorsal cortex, corpus callosum, hippocampus and cerebellum (PubMed:31297047).
Neonatal neurogenesis and gliogenesis are deeply impaired (PubMed:31297047).

Variants

We now provide the "Disease & Variants" viewer in its own tab.

The viewer provides 35 variants from UniProt as well as other sources including ClinVar and dbSNP.

Go to variant viewer

PTM/Processing

Features

Showing features for transit peptide, chain, modified residue.

TypeIDPosition(s)Description
Transit peptide1-33Mitochondrion
ChainPRO_000001998234-463NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial
Modified residue62N6-acetyllysine
Modified residue118Symmetric dimethylarginine

Post-translational modification

Dimethylation at Arg-118 by NDUFAF7 takes place after NDUFS2 assembles into the complex I, leading to stabilize the early intermediate complex.

Keywords

Proteomic databases

PTM databases

Expression

Gene expression databases

Interaction

Subunit

Core subunit of respiratory chain NADH dehydrogenase (Complex I) which is composed of 45 different subunits (PubMed:38575788).
Component of the iron-sulfur (IP) fragment of the enzyme. Interacts with NDUFAF3. Interacts with NDUFAF7 (By similarity).
Interacts with CERS2 (PubMed:32279995).
View interactors in UniProtKB
View CPX-266 in Complex Portal

Protein-protein interaction databases

Miscellaneous

Family & Domains

Sequence similarities

Belongs to the complex I 49 kDa subunit family.

Keywords

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Sequence processing
    The displayed sequence is further processed into a mature form.
  • Length
    463
  • Mass (Da)
    52,626
  • Last updated
    2001-12-01 v1
  • Checksum
    989934D73F5C8D27
MAALRALRCLRGVGAPVLRPGSGIRLPSQPSRGARQWQPDIEWAEQFSGAVMYPSKETAHWKPPPWNDVDILKEKAVTNMTLNFGPQHPAAHGVLRLVLELSGEMVRKCDPHIGLLHRGTEKLIEYKTYLQALPYFDRLDYVSMMCNEQAYSIAVEKLLNIQPPPRAQWIRVLFGEITRILNHIMAVTTHALDIGAMTPFFWMFEEREKMFEFYERVSGARMHAAYIRPGGVHQDLPLGLLDDIYEFSKNFSLRIDEVEEMLTNNRIWRNRTVDIGVVTAEDALNYGFSGVMLRGSGIQWDLRKTQPYDVYDQVEFDVPIGSRGDCYDRYLCRVEEMRQSLRIIEQCLNKMPPGEIKVDDAKVSPPKRAEMKTSMESLIHHFKLYTEGYQVPPGATYTAIEAPKGEFGVYLVSDGSSRPYRCKIKAPGFAHLAGLDKMSKGHMLADVVAIIGTQDIVFGEIDR

Computationally mapped potential isoform sequences

There are 3 potential isoforms mapped to this entry

View all
EntryEntry nameGene nameLength
A0A0A6YW30A0A0A6YW30_MOUSENdufs2231
A0A0A6YXD3A0A0A6YXD3_MOUSENdufs2145
D3YXT0D3YXT0_MOUSENdufs2437

Sequence caution

The sequence AAH03898.1 differs from that shown. Reason: Erroneous initiation

Features

Showing features for sequence conflict.

TypeIDPosition(s)Description
Sequence conflict1in Ref. 2; AAH03898
Sequence conflict2in Ref. 1; BAE30656/BAE31936
Sequence conflict81in Ref. 1; BAE27028
Sequence conflict186in Ref. 1; BAE27028

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
AK078474
EMBL· GenBank· DDBJ
BAC37293.1
EMBL· GenBank· DDBJ
mRNA
AK146269
EMBL· GenBank· DDBJ
BAE27028.1
EMBL· GenBank· DDBJ
mRNA
AK150431
EMBL· GenBank· DDBJ
BAE29554.1
EMBL· GenBank· DDBJ
mRNA
AK151746
EMBL· GenBank· DDBJ
BAE30656.1
EMBL· GenBank· DDBJ
mRNA
AK153364
EMBL· GenBank· DDBJ
BAE31936.1
EMBL· GenBank· DDBJ
mRNA
AK165426
EMBL· GenBank· DDBJ
BAE38181.1
EMBL· GenBank· DDBJ
mRNA
BC003898
EMBL· GenBank· DDBJ
AAH03898.1
EMBL· GenBank· DDBJ
mRNA Different initiation
BC016097
EMBL· GenBank· DDBJ
AAH16097.1
EMBL· GenBank· DDBJ
mRNA

Genome annotation databases

Similar Proteins

Disclaimer

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