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Product Details
PA5-139790
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant protein corresponding to Human BCKDHB. Recombinant protein control fragment (Product #RP-108805).
Conjugate
Form
Liquid
Concentration
0.05 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.2, with 40% glycerol
Contains
0.02% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
AB_2926792
Product Specific Information
Gently mix before use. Optimal concentrations and conditions for each application should be determined by the user.
Immunogen sequence: TIIPWDVDTI CKSVIKTGRL LISHEAPLTG GFASEISSTV QEECFLNLEA PISRVCGYDT PFPHIFEPFY IPDKWKCYDA L
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKD BCKDH E1-beta; BCKDE1B; 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-beta subunit of the branched-chain complex; testis secretory sperm-binding protein Li 240mP
Gene Aliases: BCKDE1B; BCKDH E1-beta; BCKDHB; E1B
UniProt ID: (Human) P21953
Entrez Gene ID: (Human) 594
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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