BCKDHB Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ELISA, WB
Recommend Dilution	WB,1:1000 - 1:5000 ELISA,Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
Reactivity	Mouse, Rat
Modifications	Unmodified
Host	Rabbit
Clonality	Polyclonal
Isotype	IgG
Formulation	Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Concentration	lot specific
Purification	Affinity purification
Conjugation	Unconjugated
Storage Condition	Store at -20℃. Avoid freeze / thaw cycles.
Predicted Protein Size	43kDa
Gene Name	branched chain keto acid dehydrogenase E1, beta polypeptide
Database Link	UniProt ID P21953
Background	This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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 multiple transcript variants.
Synonyms	dJ279A18.1; E1B; FLJ17880
Reference Data

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