UniProt functional annotation for Q9C8L4



	UniProt functional annotation for Q9C8L4

UniProt code: Q9C8L4.

Organism: Arabidopsis thaliana (Mouse-ear cress).

Taxonomy: Eukaryota; Viridiplantae; Streptophyta; Embryophyta; Tracheophyta; Spermatophyta; Magnoliopsida; eudicotyledons; Gunneridae; Pentapetalae; rosids; malvids; Brassicales; Brassicaceae; Camelineae; Arabidopsis.

Function: Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) gives 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. Required for normal endosperm development in seed, and thereby also required for normal embryo development. {ECO:0000269|PubMed:22786886}.

Catalytic activity: Reaction=H2O + O2 + S-sulfanylglutathione = glutathione + 2 H(+) + sulfite; Xref=Rhea:RHEA:12981, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:17359, ChEBI:CHEBI:57925, ChEBI:CHEBI:58905; EC=1.13.11.18; Evidence={ECO:0000269|PubMed:22786886};

Cofactor: Name=Fe(2+); Xref=ChEBI:CHEBI:29033; Evidence={ECO:0000269|PubMed:16929096}; Note=Binds 1 Fe(2+) ion per subunit. {ECO:0000269|PubMed:16929096};

Subunit: Homodimer. {ECO:0000269|PubMed:16929096}.

Subcellular location: Mitochondrion {ECO:0000269|PubMed:22786886}.

Disruption phenotype: Embryonic lethality at the early heart stage, due to defects in endosperm development. {ECO:0000269|PubMed:22786886}.

Similarity: Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family. {ECO:0000305}.

Sequence caution: Sequence=AAB17995.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305}; Sequence=AAF78432.1; Type=Erroneous gene model prediction; Evidence={ECO:0000305}; Sequence=AAG51989.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305}; Sequence=AAK96491.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305}; Sequence=AAL25570.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305};

Annotations taken from UniProtKB at the EBI.


Organism:		Arabidopsis thaliana (Mouse-ear cress).
Taxonomy:		Eukaryota; Viridiplantae; Streptophyta; Embryophyta; Tracheophyta; Spermatophyta; Magnoliopsida; eudicotyledons; Gunneridae; Pentapetalae; rosids; malvids; Brassicales; Brassicaceae; Camelineae; Arabidopsis.



Function:		Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) gives 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. Required for normal endosperm development in seed, and thereby also required for normal embryo development. {ECO:0000269\|PubMed:22786886}.


Catalytic activity:		Reaction=H2O + O2 + S-sulfanylglutathione = glutathione + 2 H(+) + sulfite; Xref=Rhea:RHEA:12981, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:17359, ChEBI:CHEBI:57925, ChEBI:CHEBI:58905; EC=1.13.11.18; Evidence={ECO:0000269\|PubMed:22786886};
Cofactor:		Name=Fe(2+); Xref=ChEBI:CHEBI:29033; Evidence={ECO:0000269\|PubMed:16929096}; Note=Binds 1 Fe(2+) ion per subunit. {ECO:0000269\|PubMed:16929096};
Subunit:		Homodimer. {ECO:0000269\|PubMed:16929096}.
Subcellular location:		Mitochondrion {ECO:0000269\|PubMed:22786886}.
Disruption phenotype:		Embryonic lethality at the early heart stage, due to defects in endosperm development. {ECO:0000269\|PubMed:22786886}.
Similarity:		Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family. {ECO:0000305}.
Sequence caution:		Sequence=AAB17995.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305}; Sequence=AAF78432.1; Type=Erroneous gene model prediction; Evidence={ECO:0000305}; Sequence=AAG51989.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305}; Sequence=AAK96491.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305}; Sequence=AAL25570.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305};