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

ETFA, CT (ETFA, Electron transfer flavoprotein subunit alpha, mitochondrial) (MaxLight 550)

Cat. Num. AAA6296917

• Gene Names: ETFA; EMA; GA2; MADD
• Reactivity: Human, Mouse
• Applications: Immunohistochemistry, Western Blot, Flow Cytometry, Functional Assay
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: ETFA; Polyclonal Antibody; CT (ETFA; Electron transfer flavoprotein subunit alpha; mitochondrial) (MaxLight 550); mitochondrial; anti-ETFA antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ETFA. Species Crossreactivity: mouse
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight550.

• Applicable Applications for anti-ETFA antibody: Immunohistochemistry (IHC) Paraffin, Western Blot (WB), Flow Cytometry (FC/FACS), FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 276-304 in the C-terminal region of human ETFA. Species sequence homology: bovine, monkey, rat
• 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 in K562 cell line and mouse liver tissue lysates (35ug/lane) usingMBS6012804. This demonstrates that MBS6012804 detected the ETFA protein (arrow).)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin fixed and paraffin embedded human skeletal muscle using MBS6012804 followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of MBS6012804 for immunohistochemistry.)

Product Categories/Family for anti-ETFA antibody

Antibodies; Mitochondrial Proteins

References

1. Ohkuma, A., et al. Muscle Nerve 39(3):333-342(2009). 2. Chiong, M.A., et al. Mol. Genet. Metab. 92 (1-2), 109-114 (2007). 3. Olsen, J.V., et al. Cell 127(3):635-648(2006). 4. Olsen, J.V., et al. Cell 127(3):635-648(2006). 5. Schiff, M., et al. Mol. Genet. Metab. 88(2):153-158(2006).

NCBI and Uniprot Product Information

• NCBI GI #: 4503607
• NCBI GeneID: 2108
• NCBI Accession #: NP_000117.1
• NCBI GenBank Nucleotide #: NM_000126.3
• UniProt Accession #: P13804; Q53XN3; B4DT43
• Molecular Weight: 35,080 Da
• NCBI Official Full Name: electron transfer flavoprotein subunit alpha, mitochondrial isoform a
• NCBI Official Synonym Full Names: electron-transfer-flavoprotein, alpha polypeptide
• NCBI Official Symbol: ETFA
• NCBI Official Synonym Symbols: EMA; GA2; MADD
• NCBI Protein Information: electron transfer flavoprotein subunit alpha, mitochondrial; alpha-ETF; glutaric aciduria II; electron transfer flavoprotein alpha-subunit; electron transfer flavoprotein, alpha polypeptide
• UniProt Protein Name: Electron transfer flavoprotein subunit alpha, mitochondrial
• Protein Family: Electron transfer flavoprotein
• UniProt Gene Name: ETFA
• UniProt Synonym Gene Names: Alpha-ETF
• UniProt Entry Name: ETFA_HUMAN

NCBI Description

ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

ETFA: The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl- CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Defects in ETFA are the cause of glutaric aciduria type 2A (GA2A); also known as glutaricaciduria IIA. GA2A is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Belongs to the ETF alpha-subunit/FixB family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 15q23-q25

Cellular Component: mitochondrion; mitochondrial matrix

Molecular Function: FAD binding; electron carrier activity; oxidoreductase activity

Biological Process: cellular metabolic process

Disease: Multiple Acyl-coa Dehydrogenase Deficiency