2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody

2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
292.5€ (80 µl)

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Name
2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
Category
Primary Antibodies
Provider
Abbexa
Reference
abx025837
Tested Applications
ELISA, WB, IHC, IF/ICC, FCM

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.

Documentos del producto

Instrucciones
Data sheet
Descargar

Especificaciones del producto

Category
Primary Antibodies
Immunogen Target
Target: 2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB)
Immunogen: KLH-conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
Host
Rabbit
Reactivity
Human
Recommended Dilution
WB: 1/1000, IHC-P: 1/500, IF/ICC: 1/25, FCM: 1/10 - 1/50. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user.
Clonality
Polyclonal
Conjugation
Unconjugated
Isotype
IgG
Observed MW
Calculated MW: 43.1 kDa
Purification
Purified through a protein A column, followed by peptide affinity purification.
Size 1
80 µl
Size 2
400 µl
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF/ICC, FCM
Buffer
PBS containing 0.09% sodium azide.
Availability
Shipped within 5-10 working days.
Storage
Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Dry Ice
No
UniProt ID
P21953
Background
Antibody anti-BCKDHB
Status
RUO
Note
THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION.

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