Anti-BCKDHB antibody (ab201225)
- Datasheet
- References (1)
- Protocols
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.
Properties
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Form
Lyophilised: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
References
This product has been referenced in:
- Leandro J et al. Mild inborn errors of metabolism in commonly used inbred mouse strains. Mol Genet Metab N/A:N/A (2019). Read more (PubMed: 30709776) »
