ACADSB sirna

ACADSB siRNA (Human)

Cat. Num. AAA827820

• Gene Names: ACADSB; ACAD7; SBCAD; 2-MEBCAD
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: ACADSB; ACADSB siRNA (Human); 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 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: ACADSB siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 330

• Applicable Applications for ACADSB sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human ACADSB gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for ACADSB sirna

siRNA to inhibit ACADSB expression using RNA interference

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
• 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: Lipid Metabolism - fatty acid; EC 1.3.8.5; Mitochondrial; 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