Anti-ETHE1 Antibody (CAB10142)

Antibody Name:	Anti-ETHE1 Antibody
Antibody SKU:	CAB10142
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 8-245 of human ETHE1 (NP_055112.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:200 - 1:1000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	SW480, Mouse liver, Mouse kidney, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 8-245 of human ETHE1 (NP_055112.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	VARR QLSQ RGGS GAPI LLRQ MFEP VSCT FTYL LGDR ESRE AVLI DPVL ETAP RDAQ LIKE LGLR LLYA VNTH CHAD HITG SGLL RSLL PGCQ SVIS RLSG AQAD LHIE DGDS IRFG RFAL ETRA SPGH TPGC VTFV LNDH SMAF TGDA LLIR GCGR TDFQ QGCA KTLY HSVH EKIF TLPG DCLI YPAH DYHG FTVS TVEE ERTL NPRL TLSC EEFV KIMG NLNL PKPQ QIDF AVPA NM
Gene ID:	23474
Uniprot:	O95571
Cellular Location:	Cytoplasm, Mitochondrion matrix, Nucleus
Calculated MW:	27kDa
Observed MW:	28kDa

Synonyms:

ETHE1, HSCO, YF13H12

Background:

This gene encodes a member of the metallo beta-lactamase family of iron-containing proteins involved in the mitochondrial sulfide oxidation pathway. The encoded protein catalyzes the oxidation of a persulfide substrate to sulfite. Certain mutations in this gene cause ethylmalonic encephalopathy, an infantile metabolic disorder affecting the brain, gastrointestinal tract and peripheral vessels. Alternative splicing results in multiple transcript variants encoding different isoforms.

UniProt Protein Function:	ETHE1: Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53- induced apoptosis by preventing nuclear localization of RELA. Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE). EE is an 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. Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.
UniProt Protein Details:	Protein type:Mitochondrial; Hydrolase; EC 1.13.11.18 Chromosomal Location of Human Ortholog: 19q13.31 Cellular Component: cytoplasm; mitochondrial matrix; mitochondrion; nucleoplasm Molecular Function:iron ion binding; sulfur dioxygenase activity Biological Process: glutathione metabolic process Disease: Encephalopathy, Ethylmalonic
NCBI Summary:	This gene encodes a member of the metallo beta-lactamase family of iron-containing proteins involved in the mitochondrial sulfide oxidation pathway. The encoded protein catalyzes the oxidation of a persulfide substrate to sulfite. Certain mutations in this gene cause ethylmalonic encephalopathy, an infantile metabolic disorder affecting the brain, gastrointestinal tract and peripheral vessels. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Mar 2016]
UniProt Code:	O95571
NCBI GenInfo Identifier:	73919341
NCBI Gene ID:	23474
NCBI Accession:	O95571.2
UniProt Secondary Accession:	O95571,Q96HR0, Q9H001,
UniProt Related Accession:	O95571
Molecular Weight:	27,873 Da
NCBI Full Name:	Persulfide dioxygenase ETHE1, mitochondrial
NCBI Synonym Full Names:	ETHE1, persulfide dioxygenase
NCBI Official Symbol:	ETHE1
NCBI Official Synonym Symbols:	HSCO; YF13H12
NCBI Protein Information:	persulfide dioxygenase ETHE1, mitochondrial
UniProt Protein Name:	Persulfide dioxygenase ETHE1, mitochondrial
UniProt Synonym Protein Names:	Ethylmalonic encephalopathy protein 1; Hepatoma subtracted clone one protein; Sulfur dioxygenase ETHE1
Protein Family:	Persulfide dioxygenase
UniProt Gene Name:	ETHE1
UniProt Entry Name:	ETHE1_HUMAN

	Western blot analysis of extracts of various cell lines, using ETHE1 antibody (CAB10142) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
	Immunohistochemistry of paraffin-embedded human colon carcinoma using ETHE1 antibody (CAB10142) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse kidney using ETHE1 antibody (CAB10142) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of U-2 OS cells using ETHE1 Polyclonal Antibody (CAB10142) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)