Western Blot (WB)
(BCKDHB Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the BCKDHB antibody detected the BCKDHB protein (arrow).)
Rabbit anti-Human BCKDHB Polyclonal Antibody | anti-BCKDHB antibody
BCKDHB, NT (BCKDHB, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain)
Purified by Protein A affinity chromatography.
Purified by Protein A affinity chromatography.
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500
Immunohistochemistry: 1:50-100
Flow Cytometry: 1:10-50
Immunofluorescence: 1:10-50
Western Blot (WB)
(BCKDHB Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the BCKDHB antibody detected the BCKDHB protein (arrow).)
Western Blot (WB)
(BCKDHB Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the BCKDHB antibody detected the BCKDHB protein (arrow).)
Immunohistochemistry (IHC)
(BCKDHB Antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the BCKDHB Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.)
Immunohistochemistry (IHC)
(BCKDHB Antibody (N-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the BCKDHB Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.)
Western Blot (WB)
(Western blot analysis of BCKDHB (arrow) using rabbit polyclonal BCKDHB Antibody (N-term) . 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the BCKDHB gene.)
Western Blot (WB)
(Western blot analysis of BCKDHB (arrow) using rabbit polyclonal BCKDHB Antibody (N-term) . 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the BCKDHB gene.)
Flow Cytometry (FC/FACS)
(BCKDHB Antibody (N-term) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)
Flow Cytometry (FC/FACS)
(BCKDHB Antibody (N-term) flow cytometric analysis of Hela cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of BCKDHB Antibody (N-term) with Hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)
Immunofluorescence (IF)
(Confocal immunofluorescent analysis of BCKDHB Antibody (N-term) with Hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)
NCBI and Uniprot Product Information
NCBI 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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq, Jul 2008]
Uniprot Description
Function: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Catalytic activity: 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2.
Subunit structure: Heterotetramer of 2 alpha and 2 beta chains.
Subcellular location: Mitochondrion matrix.
Involvement in disease: Maple syrup urine disease 1B (MSUD1B) [MIM:248600]: A metabolic disorder due to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. Accumulation of these 3 amino acids and their corresponding keto acids leads to encephalopathy and progressive neurodegeneration. Clinical features include mental and physical retardation, feeding problems, and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine. If untreated, maple syrup urine disease can lead to seizures, coma, and death. The disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy or childhood and are typically milder, but they still involve developmental delay and other medical problems if not treated.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.12 Ref.13 Ref.14
Research Articles on BCKDHB
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Product Notes
The BCKDHB bckdhb (Catalog #AAA648115) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The BCKDHB, NT (BCKDHB, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's BCKDHB can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS), Immunofluorescence (IF). Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, Flow Cytometry, ELISA Dilution: ELISA: 1:1,000 Western Blot: 1:100-500 Immunohistochemistry: 1:50-100 Flow Cytometry: 1:10-50 Immunofluorescence: 1:10-50. Researchers should empirically determine the suitability of the BCKDHB bckdhb for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "BCKDHB, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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