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You are here:Home » Antibodies » Abs to Mitochondrial Proteins » Anti -BCKDHB, NT (BCKDHB, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain)

Anti -BCKDHB, NT (BCKDHB, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial,
Branched-chain alpha-keto acid dehydrogenase E1 component beta chain)

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified E B IH FC IF
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.
Catalog #032452
ApplicationsSuitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, Flow Cytometry, ELISA
Recommended DilutionELISA: 1:1,000
Western Blot: 1:100-500
Immunohistochemistry: 1:50-100
Flow Cytometry: 1:10-50
Immunofluorescence: 1:10-50
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypePolyclonal
IsotypeIgG
HostRabbit
SourceHuman
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
PurityPurified by Protein A affinity chromatography.
ImmunogenBCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 44-74 amino acids from the N-terminal region of human BCKDHB.
SpecificityHuman
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.
Alternate namesBCKDHB, 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain


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