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Anti-ETHE1 Antibody

品牌：Antibodies

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规格：100µl

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Anti-ETHE1 Antibody

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Overview

Name:	Anti-ETHE1 Antibody See all ETHE1 primary antibodies
Description:	Rabbit polyclonal antibody to ETHE1
Specificity:	The antibody detects endogenous levels of total ETHE1 protein.
Applications:	WB, IHC
Reactivity:	Human, Mouse
Immunogen:	Fusion protein corresponding to residues near the N terminal of human ethylmalonic encephalopathy 1
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Purification:	Antigen affinity purification.
Concentration:	1.6mg / ml
Formulation:	Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Storage:	Store at -20?C

Target

Function:	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. 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 (PubMed:12398897).
Tissue Specificity:	Ubiquitously expressed.
Involvement in Disease:	Ethylmalonic encephalopathy: Autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.
Sequence Similarities:	Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.
Cellular Location:	Cytoplasm. Nucleus. Mitochondrion matrix.
Database Links:	Entrez Gene: 23474?Human Entrez Gene: 66071?Mouse Omim: 608451?Human SwissProt: O95571?Human SwissProt: Q9DCM0?Mouse Unigene: 7486?Human Unigene: 29553?Mouse
Synonyms:	ETHE1 Antibody ETHE1 protein, mitochondrial precursor Antibody ETHE1_HUMAN Antibody ethylmalonic encephalopathy 1 Antibody Ethylmalonic encephalopathy protein 1 Antibody hepatoma subtracted clone one Antibody Hepatoma subtracted clone one protein Antibody HSCO Antibody mitochondrial Antibody Protein ETHE1 Antibody Protein ETHE1 mitochondrial Antibody YF13H12 Antibody
Information:	Target information shown above is from the UniProt Consortium.