BCKDHB sirna

BCKDHB siRNA (Human)

Cat. Num. AAA8211212

• Gene Names: BCKDHB; E1B; dJ279A18.1
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: BCKDHB; BCKDHB siRNA (Human); 2-oxoisovalerate dehydrogenase subunit beta mitochondrial; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; BCKDE1B; BCKDH E1-beta; BCKDHB sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: BCKDHB 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: 218

• Applicable Applications for BCKDHB 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 BCKDHB 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 BCKDHB sirna

siRNA to inhibit BCKDHB expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 4557353
• NCBI GeneID: 594
• NCBI Accession #: NP_000047.1
• NCBI GenBank Nucleotide #: NM_000056.3
• UniProt Accession #: P21953; Q5T2J3; Q9BQL0
• Molecular Weight: 23,550 Da
• NCBI Official Full Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
• NCBI Official Synonym Full Names: branched chain keto acid dehydrogenase E1, beta polypeptide
• NCBI Official Symbol: BCKDHB
• NCBI Official Synonym Symbols: E1B; dJ279A18.1
• NCBI Protein Information: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
• UniProt Protein Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
• UniProt Gene Name: BCKDHB
• UniProt Synonym Gene Names: BCKDE1B; BCKDH E1-beta
• UniProt Entry Name: ODBB_HUMAN

NCBI Description

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq, Jul 2008]

Uniprot Description

BCKDHB: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.

Protein type: Mitochondrial; EC 1.2.4.4; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 6q14.1

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrion; mitochondrial matrix

Molecular Function: protein binding; alpha-ketoacid dehydrogenase activity; protein complex binding; carboxy-lyase activity; 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity

Biological Process: response to cAMP; response to glucocorticoid stimulus; branched chain family amino acid catabolic process; response to nutrient

Disease: Maple Syrup Urine Disease