Rabbit anti-Human, Mouse ETHE1 Polyclonal Antibody | anti-ETHE1 antibody

ETHE1, ID (Protein ETHE1, Mitochondrial, Ethylmalonic Encephalopathy Protein 1, Hepatoma Subtracted Clone One Protein, HSCO) (MaxLight 550)

Cat. Num. AAA6475051

• Gene Names: ETHE1; HSCO; YF13H12
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: ETHE1; Polyclonal Antibody; ID (Protein ETHE1; Mitochondrial; Ethylmalonic Encephalopathy Protein 1; Hepatoma Subtracted Clone One Protein; HSCO) (MaxLight 550); anti-ETHE1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ETHE1. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight550.

• Applicable Applications for anti-ETHE1 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:50-1:100; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the center region of human ETHE1 (KLH).
• Conjugate: MaxLight550
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight550 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of ETHE1 antibody (Center) in mouse kidney tissue lysates (35ug/lane). ETHE1 (arrow) was detected using the purified Pab.)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of 293 cells using ETHE1 Antibody (Center)(bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Immunohistochemistry (IHC)

(ETHE1 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human colon carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the ETHE1 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)

Western Blot (WB)

(Western blot analysis of ETHE1 (arrow) using rabbit polyclonal ETHE1 Antibody (Center). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the ETHE1 gene.)

Product Categories/Family for anti-ETHE1 antibody

Antibodies; Enzymes

References

1. Tiranti, V., Nat. Med. 15(2), 200-205 (2009). 2. Mineri, R., J. Med. Genet. 45(7), 473-478 (2008).

NCBI and Uniprot Product Information

• NCBI GI #: 41327741
• NCBI GeneID: 23474
• NCBI Accession #: NP_055112
• NCBI GenBank Nucleotide #: NM_014297.4
• UniProt Accession #: O95571; Q96HR0; Q9H001
• Molecular Weight: 29.1 kDa (267aa) confirmed by MALDI-TOF
• NCBI Official Full Name: persulfide dioxygenase ETHE1, mitochondrial isoform 1
• NCBI Official 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
• Protein Family: Persulfide dioxygenase
• UniProt Gene Name: ETHE1
• UniProt Synonym Gene Names: HSCO
• UniProt Entry Name: ETHE1_HUMAN

NCBI Description

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 Description

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.

Protein type: Hydrolase; Mitochondrial; EC 1.13.11.18

Chromosomal Location of Human Ortholog: 19q13.31

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; cytoplasm

Molecular Function: iron ion binding; sulfur dioxygenase activity

Biological Process: glutathione metabolic process; sulfur amino acid metabolic process; sulfur amino acid catabolic process

Disease: Encephalopathy, Ethylmalonic