BCKDHB Antibody - BSA Free

Western Blot: BCKDHB Antibody - BSA Free [NBP2-92027] - Western blot analysis of various lysates, using BCKDHB antibody at 1:2000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 60s.

• Reactivity: Mu, Rt
• Applications: WB
• Clonality: Polyclonal
• Host: Rabbit
• Conjugate: Unconjugated
• Format: BSA Free

BCKDHB Antibody - BSA Free Summary

• Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871.1). RSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
• Isotype: IgG
• Clonality: Polyclonal
• Host: Rabbit
• Gene: BCKDHB
• Purity: Affinity purified

Applications/Dilutions

• Dilutions:
  • Western Blot 1:1000 - 1:5000

Packaging, Storage & Formulations

• Storage: Store at -20C. Avoid freeze-thaw cycles.
• Buffer: PBS (pH 7.3), 50% glycerol
• Preservative: 0.02% Sodium Azide
• Purity: Affinity purified

Alternate Names for BCKDHB Antibody - BSA Free

• 2-oxoisovalerate dehydrogenase beta subunit
• 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
• BCKDE1B
• BCKDH E1-beta
• branched chain alpha-ketoacid dehydrogenase E1-beta subunit
• branched chain keto acid dehydrogenase E1, beta polypeptide
• Branched-chain alpha-keto acid dehydrogenase E1 component beta chain
• dJ279A18.1
• E1B
• E1b-beta subunit of the branched-chain complex
• EC 1.2.4.4
• FLJ17880

Background

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, andfunctions 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 physicalretardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different3' non-coding regions, but encoding the same isoform. (provided by RefSeq)

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: BCKDHB