Human ETFB Recombinant Protein (RPPB5884)

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SKU:
RPPB5884
Product type:
Recombinant Protein
Size:
10ug
Species:
Human
Target:
ETFB
Synonyms:
Electron-transfer-flavoprotein beta polypeptide
Synonyms:
MADD
Synonyms:
beta-ETF
Source:
Escherichia Coli
Uniprot:
P38117
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Description

Product Name:Human ETFB Recombinant Protein
Product Code:RPPB5884
Size:10µg
Species:Human
Target:ETFB
Synonyms:Electron-transfer-flavoprotein beta polypeptide, MADD, beta-ETF.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The ETFB solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 40% glycerol and 0.1M NaCl.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MAELRVLVAV KRVIDYAVKI RVKPDRTGVV TDGVKHSMNP FCEIAVEEAV RLKEKKLVKE VIAVSCGPAQ CQETIRTALA MGADRGIHVE VPPAEAERLG PLQVARVLAK LAEKEKVDLV LLGKQAIDDD CNQTGQMTAG FLDWPQGTFA SQVTLEGDKLKVEREIDGGL ETLRLKLPAV VTADLRLNEP RYATLPNIMK AKKKKIEVIK PGDLGVDLTS KLSVISVEDP PQRTAGVKVE TTEDLVAKLK EIGRI

ETF is a heterodimer composed of alpha and beta subunit. ETFB protein is electron-transfer-flavoprotein, beta polypeptide that transports electrons between primary flavoprotein dehydrogenases involved in mitochondrial fatty acid and amino acid catabolism and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase.

ETFB Human Recombinant fused with a 20 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 275 amino acids (1-255a.a.) and having a molecular mass of 30.0kDa. The ETFB is purified by proprietary chromatographic techniques.

UniProt Protein Function:ETFB: 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 ETFB are the cause of glutaric aciduria type 2B (GA2B). GA2B 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 beta-subunit/FixA family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 19q13.3

Cellular Component: intracellular membrane-bound organelle; mitochondrion; mitochondrial matrix

Molecular Function:electron carrier activity

Biological Process: cellular metabolic process

Disease: Multiple Acyl-coa Dehydrogenase Deficiency

NCBI Summary:This gene encodes electron-transfer-flavoprotein, beta polypeptide, which shuttles electrons between primary flavoprotein dehydrogenases involved in mitochondrial fatty acid and amino acid catabolism and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. The gene deficiencies have been implicated in type II glutaricaciduria. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:P38117
NCBI GenInfo Identifier:62420877
NCBI Gene ID:2109
NCBI Accession:NP_001014763.1
UniProt Secondary Accession:P38117,Q6IBH7, Q71RF6, Q9Y3S7, B3KNY2,
UniProt Related Accession:P38117
Molecular Weight:37,566 Da
NCBI Full Name:electron transfer flavoprotein subunit beta isoform 2
NCBI Synonym Full Names:electron-transfer-flavoprotein, beta polypeptide
NCBI Official Symbol:ETFB  
NCBI Official Synonym Symbols:MADD; FP585  
NCBI Protein Information:electron transfer flavoprotein subunit beta; beta-ETF; electron transfer flavoprotein beta subunit; electron transfer flavoprotein beta-subunit; electron transfer flavoprotein, beta polypeptide; electron-transferring-flavoprotein, beta polypeptide
UniProt Protein Name:Electron transfer flavoprotein subunit beta
Protein Family:Electron transfer flavoprotein
UniProt Gene Name:ETFB  
UniProt Entry Name:ETFB_HUMAN
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