BCKDHB Rabbit mAb,ABclonal,A24618

Reactivity

Human, Mouse, Rat

Application

WB, IHC-P, ELISA

Conjugate

Unconjugated

Platform ID

BAB793509721

ABclonal

Headquarters

500W Cummings Park, Ste. 6500 Woburn, MA 01801

ABclonal

Contact

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Email:

[email protected]

Product Specifications
Scientific Background
Synonyms

Specifications

NameBCKDHB Rabbit mAb
Cat. No.A24618
Accession NumberP21953
Gene ID (Entrez)594
IsotypeIgG
ReactivityHuman, Mouse, Rat
ConjugationUnconjugated
ApplicationWB, IHC-P, ELISA
Working DilutionsWB:1:1000 - 1:8000 | IHC-P:1:200 - 1:800 | ELISA:Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
Clone NumberARC62319
Molecular Weight37 kDa
ImmunogenRecombinant protein (or fragment).This information is considered to be commercially sensitive.
PurityAffinity purification
Appearance/FormLiquid
StorageStore at -20℃. Avoid freeze / thaw cycles.; Buffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide (as specified on the Certificate of Analysis), pH 7.3.
Regulatory StatusResearch Use Only

Scientific 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

E1B; BCKDE1B; BCKDH E1-beta; BCKDHB

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