BCKDHB Antibody
| Code | Size | Price |
|---|
| PSI-55-327-400ul | 400ul | £626.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: Rabbit Ig
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species: Human
Applications:
- Fluorescence-activated cell sorting (FACS)
- Immunofluorescence (IF)
- Immunohistochemistry- Paraffin Embedded (IHC-P)
- Western Blot (WB)
Storage:
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Documents
Further Information
Additional Names:
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB
Application Note:
For WB starting dilution is: 1:1000
For IHC-P starting dilution is: 1:50~100
For FACS starting dilution is: 1:10~50
For IF starting dilution is: 1:10~50
For IHC-P starting dilution is: 1:50~100
For FACS starting dilution is: 1:10~50
For IF starting dilution is: 1:10~50
Background:
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.
Background References:
- Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :
- Gorzelany, K., et al. Turk. J. Pediatr. 51(2):97-102(2009)
- Quental, S., et al. Mol. Genet. Metab. 94(2):148-156(2008)
- Kang, H., et al. Fertil. Steril. 89(3):728-731(2008)
Buffer:
Supplied in PBS with 0.09% (W/V) sodium azide.
Concentration:
batch dependent
Conjugate:
Unconjugated
DISCLAIMER:
Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Immunogen:
This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
NCBI Gene ID #:
594
NCBI Official Name:
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
NCBI Official Symbol:
BCKDHB
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
43 kDa
Protein Accession #:
P21953
Protein GI Number:
129034
Purification:
This antibody is purified through a protein A column, followed by peptide affinity purification.
Research Area:
Obesity,Signal Transduction
Swissprot #:
P21953
User NOte:
Optimal dilutions for each application to be determined by the researcher.