Loading...

Skip to main content

Call us on + 1 (800) 604-9114 for more information about our products

Looking for specific datasheet Manual/COA/MSDS?
Request a Manual/COA/MSDS

Interested to get a quote about our products?
Request a Quote

Western Blot (WB) (Western blot analysis of BCKDHA Antibody (C-term) in Jurkat cell line lysates (35ug/lane). BCKDHA (arrow) was detected using the purified Pab.)

Rabbit anti-Human BCKDHA Polyclonal Antibody | anti-BCKDHA antibody

BCKDHA, CT (2-oxoisovalerate Dehydrogenase Subunit alpha, Mitochondrial, Branched-chain alpha-keto Acid Dehydrogenase E1 Component alpha Chain, BCKDH E1-alpha, BCKDE1A) (Biotin)

Gene Names
BCKDHA; MSU; MSUD1; OVD1A; BCKDE1A
Reactivity
Human
Applications
Western Blot, ELISA
Purity
Purified by Ammonium Sulfate Precipitation.
Synonyms
BCKDHA; Polyclonal Antibody; CT (2-oxoisovalerate Dehydrogenase Subunit alpha; Mitochondrial; Branched-chain alpha-keto Acid Dehydrogenase E1 Component alpha Chain; BCKDH E1-alpha; BCKDE1A) (Biotin); anti-BCKDHA antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human
Clonality
Polyclonal
Isotype
IgG
Specificity
Recognizes human BCKDHA.
Purity/Purification
Purified by Ammonium Sulfate Precipitation.
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
Applicable Applications for anti-BCKDHA antibody
Western Blot (WB), ELISA (EIA)
Application Notes
WB: 1:50-1:100
ELISA: 1:1,000
Applications are based on unconjugated antibody.
Immunogen
Synthetic peptide selected from the C-terminal region of human BCKDHA (KLH).
Conjugate
Biotin
Note
Preservative Free
Preparation and Storage
Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of BCKDHA Antibody (C-term) in Jurkat cell line lysates (35ug/lane). BCKDHA (arrow) was detected using the purified Pab.)

Western Blot (WB) (Western blot analysis of BCKDHA Antibody (C-term) in Jurkat cell line lysates (35ug/lane). BCKDHA (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human lung carcinoma reacted with BCKDHA Antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Immunohistochemistry (IHC) (Formalin-fixed and paraffin-embedded human lung carcinoma reacted with BCKDHA Antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(BCKDHA Antibody (C-term) flow cytometric analysis of Jurkat 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) (BCKDHA Antibody (C-term) flow cytometric analysis of Jurkat cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)
Product Categories/Family for anti-BCKDHA antibody
References
1. Flaschker, N., et.al., J. Inherit. Metab. Dis. 30(6), 903-909 (2007).

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
593
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
50,790 Da
NCBI Official Full Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1
NCBI Official Synonym Full Names
branched chain keto acid dehydrogenase E1, alpha polypeptide
NCBI Official Symbol
BCKDHA
NCBI Official Synonym Symbols
MSU; MSUD1; OVD1A; BCKDE1A
NCBI Protein Information
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 2-oxoisovalerate dehydrogenase (lipoamide); BCKDH E1-alpha; branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
UniProt Protein Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
UniProt Gene Name
BCKDHA
UniProt Synonym Gene Names
BCKDE1A; BCKDH E1-alpha
UniProt Entry Name
ODBA_HUMAN

NCBI Description

The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]

Uniprot Description

BCKDH E1-alpha: branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease). Catalyzes the first irreversible and rate-limiting step in leucine oxidation. Its activity is inhibited by serine phosphorylation

Protein type: EC 1.2.4.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Oxidoreductase; Lyase

Chromosomal Location of Human Ortholog: 19q13.1-q13.2

Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrion; mitochondrial matrix

Molecular Function: protein binding; alpha-ketoacid dehydrogenase activity; metal ion binding; carboxy-lyase activity; 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity

Biological Process: branched chain family amino acid catabolic process

Disease: Maple Syrup Urine Disease

Research Articles on BCKDHA

Similar Products

Product Notes

The BCKDHA bckdha (Catalog #AAA6464989) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The BCKDHA, CT (2-oxoisovalerate Dehydrogenase Subunit alpha, Mitochondrial, Branched-chain alpha-keto Acid Dehydrogenase E1 Component alpha Chain, BCKDH E1-alpha, BCKDE1A) (Biotin) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's BCKDHA can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB: 1:50-1:100 ELISA: 1:1,000 Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the BCKDHA bckdha 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 "BCKDHA, 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

If you are ready to order, navigate to Shopping Cart and get ready to checkout.

Looking for a specific manual?
Request a Manual

Request more Information

Please complete the form below and a representative will contact you as soon as possible.

Request a Manual

Please complete the form below and a representative will contact you as soon as possible.

Request a Quote

Please complete the form below and a representative will contact you as soon as possible.