Rabbit anti-Human ACADSB Polyclonal Antibody | anti-ACADSB antibody

ACADSB Antibody (Center)

Cat. Num. AAA9231906

• Gene Names: ACADSB; ACAD7; SBCAD; 2-MEBCAD
• Reactivity: Human
• Applications: Western Blot, ELISA
• Synonyms: ACADSB; Polyclonal Antibody; ACADSB Antibody (Center); 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; anti-ACADSB antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Ig
• Specificity: This ACADSB antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 239-273 amino acids from the Central region of human ACADSB.
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Sequence Length: 432

• Applicable Applications for anti-ACADSB antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:1000
• Antigen Source: Human
• Antigen Region: 239-273
• Antigen Type: Synthetic Peptide
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Western blot analysis of lysates from human liver tissue lysate,HepG2 cell line (from left to right), using ACADSB Antibody (Center). It was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody.Lysates at 20ug per lane.)

Related Product Information for anti-ACADSB antibody

Description: Purified Rabbit Polyclonal Antibody (Pab)

Background: 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.

References

Rozen R.,et al.Genomics 24:280-287(1994). Andresen B.S.,et al.Am. J. Hum. Genet. 67:1095-1103(2000). Ota T.,et al.Nat. Genet. 36:40-45(2004). Deloukas P.,et al.Nature 429:375-381(2004). Mural R.J.,et al.Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases.

NCBI and Uniprot Product Information

• NCBI GI #: 1168283
• NCBI GeneID: 36
• UniProt Accession #: P45954
• Molecular Weight: 47485
• 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
• UniProt Protein Name: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
• Protein Family: Short/branched chain specific 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: Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.5; Lipid Metabolism - fatty acid; Mitochondrial

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