anti-Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) Antikörper

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. Zusätzlich bieten wir Ihnen BCKDHA Proteine (10) und BCKDHA Kits (5) und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
BCKDHA 593 P12694
BCKDHA 12039  
BCKDHA 25244 P11960
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Top anti-BCKDHA Antikörper auf antikoerper-online.de

Showing 10 out of 22 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Rind (Kuh) Kaninchen Unkonjugiert IHC, WB WB Suggested Anti-BCKDHA Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:312500Positive Control: MCF7 cell lysate 100 μL Anmelden zum Anzeigen 6 bis 8 Tage
358,51 €
Details
Human Kaninchen Unkonjugiert EIA, WB 0.4 mL Anmelden zum Anzeigen 7 bis 9 Tage
407,00 €
Details
Human Kaninchen Unkonjugiert FACS, IHC (p), WB Western blot analysis of BCKDHA Antibody (C-term) (ABIN390811) in Jurkat cell line lysates (35 µg/lane). BCKDHA (arrow) was detected using the purified polyclonal antibody. 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. 400 μL Anmelden zum Anzeigen 14 bis 16 Tage
335,24 €
Details
Human Kaninchen Unkonjugiert ICC, IF, WB Western Blot: BCKDHA Antibody [NBP1-79616] - MCF-7 whole cell lysates, concentration 0.2-1 ug/ml. Western Blot: BCKDHA Antibody [NBP1-79616] - Human Adult Placenta. 100 μL Anmelden zum Anzeigen 3 bis 6 Tage
341,00 €
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Rind (Kuh) Kaninchen Unkonjugiert WB 50 μg Anmelden zum Anzeigen 11 bis 14 Tage
653,02 €
Details
Human Kaninchen Unkonjugiert ICC, IF, IHC, IHC (p), WB Western Blot: BCKDHA Antibody [NBP2-15552] - Sample (30 ug of whole cell lysate) A: HepG2 10% SDS PAGE gel, diluted at 1:1000. Immunocytochemistry/Immunofluorescence: BCKDHA Antibody [NBP2-15552] - HeLa cells were fixed in ice-cold MeOH for 5 min. Green: BCKDHA protein stained by BCKDHA antibody [C1C3] diluted at 1:400. Blue: Hoechst 33342 staining. 0.1 mL Anmelden zum Anzeigen 3 bis 6 Tage
341,00 €
Details
Human Kaninchen Unkonjugiert ELISA, WB 50 μg Anmelden zum Anzeigen 6 bis 8 Tage
459,65 €
Details
Human Kaninchen Unkonjugiert WB Western blot analysis of extracts of various cells, using BCKDHA antibody. 100 μL Anmelden zum Anzeigen 12 Days
261,90 €
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Human Kaninchen Unkonjugiert WB BCKDHA antibody used at 1 ug/ml to detect target protein. 50 μg Anmelden zum Anzeigen 6 bis 8 Tage
495,58 €
Details
Human Kaninchen Unkonjugiert IHC, IHC (p) Immunohistochemistry: BCKDHA Antibody [NBP1-81022] - Staining of human kidney shows strong granular positivity in cells in tubules. 0.1 mL Anmelden zum Anzeigen 5 bis 8 Tage
335,00 €
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BCKDHA Antikörper nach Reaktivität, Anwendung, Klonalität and Konjugat

Attribute Applikationen Wirt Klonalität
Human , ,
,

Mouse (Murine)


Rat (Rattus)


Am meisten referenzierte anti-BCKDHA Antikörper

  1. Human Polyclonal BCKDHA Primary Antibody für ICC, IF - ABIN4894002 : Green, Wallace, Divakaruni, Phillips, Murphy, Ciaraldi, Metallo: Branched-chain amino acid catabolism fuels adipocyte differentiation and lipogenesis. in Nature chemical biology 2015 (PubMed)

  2. Human Polyclonal BCKDHA Primary Antibody für EIA, WB - ABIN452780 : Flaschker, Feyen, Fend, Simon, Schadewaldt, Wendel: Description of the mutations in 15 subjects with variant forms of maple syrup urine disease. in Journal of inherited metabolic disease 2007 (PubMed)

Weitere Antikörper gegen BCKDHA Interaktionspartner

Human Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) Interaktionspartner

  1. we identified seven novel genetic variants: p.Leu549Pro, p.Glu564*, p.Leu641Pro in MUT (zeige MUT Antikörper), p.Tyr206Cys in PCCB (zeige PCCB Antikörper), p.His194Arg, p.Val298Met in BCKDHA and p.Glu286_Met290del in BCKDHB (zeige BCKDHB Antikörper) gene. In silico and/or eukaryotic expression studies confirmed pathogenic effect of all novel genetic variants

  2. Five novel mutations in BCKDHA were identified in MSUD patients.

  3. The novel DBT (zeige DBT Antikörper) mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion indicating a possible founder mutation may be preserved.

  4. Targeted parallel sequencing revealed novel mutations in the gene BCKDHA for prenatal testing of maple syrup urine disease.

  5. Data from infant/her heterozygous parents (first cousins) suggest homozygous mutation (S144I) in BCKDHA can result in maple syrup urine disease (IA); molecular modeling suggests this missense mutation in exon 4 affects protein stability. [CASE STUDY]

  6. Case Report: functional characterization of the novel intronic nucleotide change c.288+9C>T within the BCKDHA gene in maple syrup urine disease.

  7. A list of nine primary candidate genes for T2D and five for obesity were identified in this paper. Two genes, LPL (zeige LCP1 Antikörper) and BCKDHA, were common to these two sets.

  8. BCKDHA and BCKDHB (zeige BCKDHB Antikörper) mutations might be primarily responsible for maple syrup urine disease in the Indian population.

  9. autism presenting with intellectual disability and epilepsy caused by BCKDK (zeige BCKDK Antikörper) mutations represents a potentially treatable syndrome.

  10. identified 4 novel mutations of the BCKDHA gene in 3 Korean newborns; to the best of knowledge, this is the first report of maple syrup urine disease confirmed by genetic analysis in Korea

Pig (Porcine) Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) Interaktionspartner

  1. analysis of NCF2 (zeige NCF2 Antikörper), BCKDHB (zeige BCKDHB Antikörper) and BCKDHA in pig

Mouse (Murine) Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA) Interaktionspartner

  1. Disruption of the BDK (zeige KNG1 Antikörper) gene establishes that regulation of BCKDH by phosphorylation is critically important for the regulation of oxidative disposal of branched-chain amino acids.

BCKDHA Antigen-Profil

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit BCKDHA

  • branched chain keto acid dehydrogenase E1, alpha polypeptide (BCKDHA) Antikörper
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (bckdha) Antikörper
  • branched chain keto acid dehydrogenase E1, alpha polypeptide (LOC100352048) Antikörper
  • 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) (BckdhA) Antikörper
  • branched chain ketoacid dehydrogenase E1, alpha polypeptide (Bckdha) Antikörper
  • BCKDA Antikörper
  • BCKDE1A Antikörper
  • E1a Antikörper
  • MSU Antikörper
  • MSUD1 Antikörper
  • OVD1A Antikörper
  • wu:fd20d04 Antikörper
  • zgc:110049 Antikörper

Bezeichner auf Proteinebene für BCKDHA

branched chain keto acid dehydrogenase E1, alpha polypeptide , 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial , 2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) , 2-oxoisovalerate dehydrogenase (lipoamide) , BCKDH E1-alpha , branched-chain alpha-keto acid dehydrogenase E1 component alpha chain , branched chain keto acid dehydrogenase E1 alpha polypeptide , BCKDE1A , branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) , BCKAD E1[a] , branched chain alpha-ketoacid dehydrogenase subunit E1 alpha , branched chain keto acid dehydrogenase subunit E1, alpha polypeptide

GENE ID SPEZIES
704978 Macaca mulatta
100036656 Xenopus (Silurana) tropicalis
100068678 Equus caballus
100145857 Ovis aries
100352048 Oryctolagus cuniculus
374210 Gallus gallus
3708743 Pseudoalteromonas haloplanktis TAC125
554124 Danio rerio
593 Homo sapiens
484488 Canis lupus familiaris
100142666 Sus scrofa
282149 Bos taurus
468889 Pan troglodytes
12039 Mus musculus
25244 Rattus norvegicus
Ausgewählte Anbieter für anti-BCKDHA (BCKDHA) Antikörper
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