Anti-BCKDHB antibody (ab201225)
Key features and details
- Rabbit polyclonal to BCKDHB
- Suitable for: IHC-P, WB
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-BCKDHB antibody -
Description
Rabbit polyclonal to BCKDHB -
Host species
Rabbit -
Tested applications
Suitable for: IHC-P, WBmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat, Cow
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Immunogen
Recombinant fragment corresponding to Human BCKDHB aa 181-391. (BC040139).
Sequence:TIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIE DKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQ VHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHE APLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKW KCYDALRKMIN
Database link: P21953 -
Positive control
- HeLa, 293T and Jurkat cell lysates. Human fetal stomach tissue.
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General notes
Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.
Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.
We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.
In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
Please check that this product meets your needs before purchasing. If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, as well as customer reviews and Q&As.
Properties
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Form
Lyophilized:Reconstitute in 200ul sterile H2O. -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 98% PBS, 1% BSA -
Concentration information loading... -
Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Positive Controls
Applications
Our Abpromise guarantee covers the use of ab201225 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| IHC-P | 1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. | |
| WB | 1/200 - 1/1000. Predicted molecular weight: 43 kDa. |
Target
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Function
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). -
Involvement in disease
Defects in BCKDHB are the cause of maple syrup urine disease type IB (MSUD1B) [MIM:248600]. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. -
Cellular localization
Mitochondrion matrix. - Information by UniProt
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Database links
- Entrez Gene: 282150 Cow
- Entrez Gene: 594 Human
- Entrez Gene: 12040 Mouse
- Entrez Gene: 29711 Rat
- Omim: 248611 Human
- SwissProt: P21839 Cow
- SwissProt: P21953 Human
- SwissProt: Q6P3A8 Mouse
see all -
Alternative names
- 2 oxoisovalerate dehydrogenase subunit beta mitochondrial antibody
- 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial antibody
- BCKDE1B antibody
see all
Images
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-BCKDHB antibody (ab201225)
Immunohistochemical analysis of paraffin embedded Human fetal stomach tissue labeling BCKDHB with ab201225 at 1/100 dilution.
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Western blot - Anti-BCKDHB antibody (ab201225)All lanes : Anti-BCKDHB antibody (ab201225) at 1/500 dilution
Lane 1 : HeLa cell lysate
Lane 2 : 293T cell lysate
Lane 3 : Jurkat cell lysate
Developed using the ECL technique.
Predicted band size: 43 kDa
Protocols
References (1)
ab201225 has been referenced in 1 publication.
- Leandro J et al. Mild inborn errors of metabolism in commonly used inbred mouse strains. Mol Genet Metab N/A:N/A (2019). PubMed: 30709776
