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branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) (BCKDHA) (Human) antibody

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Antigen: Branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) (BCKDHA)
Antibody Type: Polyclonal
Application: Western Blotting (WB), ELISA
Reactivity: Human
Host: Mouse
Quantity: 50 µl
Price: 258,00 €   (Plus shipping costs , €10.00 dry ice fee and VAT)
Order number: ABIN169849
Availability: Delivery in 7 to 10 days
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Additional Information:  branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) (BCKDHA) (Human) antibody

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Antigen Branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) (BCKDHA)
Gene ID 593
Immunogen BCKDHA (NP_000700, 347 a.a. ~ 446 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence: (without GST) SVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKP KPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK*
Reactivity Human
Antibody Type Polyclonal
Description Branched chain keto acid dehydrogenase E1, alpha polypeptide The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD, EC 1.2.4.4), an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric (alpha2, beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl transacylase (E2, MIM 248610), and a homodimeric dihydrolipoamide dehydrogenase (E3, MIM 238331). The reaction is irreversible and constitutes the first committed step in BCAA oxidation. The complex also contains 2 regulatory enzymes, a kinase and a phosphorylase. The BCKDHA gene encodes the alpha subunit of E1, and the BCKDHB gene (MIM 248611) encodes the beta subunit of E1. Mouse polyclonal antibody raised against a partial recombinant BCKDHA. Synonyms: MSU, MSUD1
Host Mouse

Application Details:  branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) (BCKDHA) (Human) antibody

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Application Western Blotting (WB), ELISA
Application Notes Quality Control: Antibody Reactive Against Recombinant Protein with GST tag on ELISA and Western Blot. GST tag alone is used as a negative control.
Buffer In 50% Glycerol Western Blot detection against Immunogen (37.00 KDa) .
Storage Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
Restrictions For Research Use only

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Western Blot detection against Immunogen (37.00 KDa) .

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Google Scholar Find more references for antigen BCKDHA on Google Scholar™
EMBL Harvester Search human, mouse or rat genome for antigen BCKDHA (Bioinformatic Harvester (EMBL))