Acyl-CoA Dehydrogenase Recombinant Protein | ACADSB recombinant protein

Recombinant Human Acyl-CoA Dehydrogenase, Short Chain

Cat. Num. AAA145182

• Gene Names: ACADSB; ACAD7; SBCAD; 2-MEBCAD
• Purity: Greater than 90.0% as determined by SDS-PAGE.
• Synonyms: Acyl-CoA Dehydrogenase; Recombinant Human Acyl-CoA Dehydrogenase; Short Chain; ACADSB Human; Short Chain Human Recombinant; Short/branched chain specific acyl-CoA dehydrogenase mitochondrial; SBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; ACADSB; ACAD7; ACADSB recombinant protein

• Host: E Coli
• Purity/Purification: Greater than 90.0% as determined by SDS-PAGE.
• Form/Format: The ACADSB solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.1M NaCl, 10% glycerol and 1mM DTT.; Sterile Filtered clear solution.
• Sequence: MGSSHHHHHH SSGLVPRGSH MGSHMKSSQS EALLNITNNG IHFAPLQTFT DEEMMIKSSV KKFAQEQIAP LVSTMDENSK MEKSVIQGLF QQGLMGIEVD PEYGGTGASF LSTVLVIEEL AKVDASVAVF CEIQNTLINT LIRKHGTEEQ KATYLPQLTT EKVGSFCLSE AGAGSDSFAL KTRADKEGDY YVLNGSKMWI SSAEHAGLFL VMANVDPTIG YKGITSFLVD RDTPGLHIGK PENKLGLRAS STCPLTFENV KVPEANILGQ IGHGYKYAIG SLNEGRIGIA AQMLGLAQGC FDYTIPYIKE RIQFGKRLFD FQGLQHQVAH VATQLEAARL LTYNAARLLE AGKPFIKEAS MAKYYASEIA GQTTSKCIEW MGGVGYTKDY PVEKYFRDAK IGTIYEGASN IQLNTIAKHI DAEY
• Sequence Length: 432

• Preparation and Storage: Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree 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.

Related Product Information for ACADSB recombinant protein

Description: ACADSB Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 424 amino acids (34-432) and having a molecular mass of 46.4kDa.ACADSB is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Introduction: Short/branched chain specific acyl-CoA dehydrogenase (ACADSB) belongs to the acyl-CoA dehydrogenase family of enzymes which catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. ACADSB catalyzes the degradation of L-isoleucine while having the highest affinity for (s)-2-methylbutyryl-CoA, isobutyryl-CoA and 2-methylhexanoyl-CoA as substrates. ACADSB may use valproyl-CoA as substrate and have a role in regulating the metabolic flux of valproic acid in the development of toxicity of this agent. ACADSB gene defects cause the short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), which is an autosomal recessive disorder characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine.

Product Categories/Family for ACADSB recombinant protein

Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 4501859
• NCBI GeneID: 36
• NCBI Accession #: NP_001600.1
• NCBI GenBank Nucleotide #: NM_001609.3
• UniProt Accession #: P45954; Q5SQN6; Q96CX7; B4DQ51
• Molecular Weight: 36,025 Da
• NCBI Official Full Name: short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
• NCBI Official Synonym Full Names: acyl-CoA dehydrogenase, short/branched chain
• NCBI Official Symbol: ACADSB
• NCBI Official Synonym Symbols: ACAD7; SBCAD; 2-MEBCAD
• NCBI Protein Information: short/branched chain specific acyl-CoA dehydrogenase, mitochondrial; 2-methyl branched chain acyl-CoA dehydrogenase; 2-methylbutyryl-coenzyme A dehydrogenase; acyl-Coenzyme A dehydrogenase, short/branched chain
• UniProt Protein Name: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
• Protein Family: Acyl-CoA dehydrogenase
• UniProt Gene Name: ACADSB
• UniProt Synonym Gene Names: SBCAD; 2-MEBCAD; 2-methylbutyryl-CoA dehydrogenase
• UniProt Entry Name: ACDSB_HUMAN

NCBI Description

Short/branched chain acyl-CoA dehydrogenase(ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa. [provided by RefSeq, Jul 2008]

Uniprot Description

ACADSB: Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD); also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2- methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. Belongs to the acyl-CoA dehydrogenase family.

Protein type: Mitochondrial; EC 1.3.8.5; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase

Chromosomal Location of Human Ortholog: 10q26.13

Cellular Component: mitochondrion; mitochondrial matrix

Molecular Function: acyl-CoA dehydrogenase activity; FAD binding

Biological Process: branched chain family amino acid catabolic process; fatty acid metabolic process

Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency