InnateDB: Systems Biology of the Innate Immune Response

Mus musculus Protein: Ethe1

Summary

InnateDB Protein

IDBP-155133.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

Ethe1

Protein Name

ethylmalonic encephalopathy 1

Synonyms

0610025L15Rik; Hsco;

Species

Mus musculus

Ensembl Protein

ENSMUSP00000076433

InnateDB Gene

IDBG-155131 (Ethe1)

Protein Structure

UniProt Annotation

Function

First described as a protein that can shuttle between the nucleus and the cytoplasm and suppress p53-induced apoptosis by sequestering the transcription factor RELA/NFKB3 in the cytoplasm and preventing its accumulation in the nucleus (By similarity). Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, due to the inhibition of cytochrome c oxidase. {ECO:0000250, ECO:0000269PubMed:19136963}.

Subcellular Localization

Cytoplasm {ECO:0000250}. Nucleus {ECO:0000250}. Mitochondrion matrix {ECO:0000250}.

Disease Associations

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 7 experimentally validated interaction(s) in this database.
They are also associated with 8 interaction(s) predicted by orthology.

Experimentally validated
Total	7 [view]
Protein-Protein	7 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	8 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0005506	iron ion binding
GO:0016787	hydrolase activity
GO:0050313	sulfur dioxygenase activity

Biological Process

GO:0006749	glutathione metabolic process
GO:0055114	oxidation-reduction process
GO:0070813	hydrogen sulfide metabolic process

Cellular Component

GO:0005634	nucleus
GO:0005737	cytoplasm
GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix

Protein Structure and Domains

PDB ID

MGI:1913321

InterPro

IPR001279 Beta-lactamase-like

PFAM

PF00753

PRINTS

PIRSF

SMART

SM00849

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q9DCM0

PhosphoSite

PhosphoSite-Q9DCM0

TrEMBL

UniProt Splice Variant

Entrez Gene

66071

UniGene

Mm.29553

RefSeq

NP_075643

MGI ID

MGI Symbol

Ethe1

OMIM

CCDS

CCDS20957

HPRD

IMGT

EMBL

AB049623 AK002666 BC010592 BC083162 BC094044

GenPept

AAH10592 AAH83162 AAH94044 BAB16409 BAB22271