PDHX anticorps (AA 1-300)
Aperçu rapide pour PDHX anticorps (AA 1-300) (ABIN6145384)
Antigène
Voir toutes PDHX AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
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Épitope
- AA 1-300
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Séquence
- MAASWRLGCD PRLLRYLVGF PGRRSVGLVK GALGWSVSRG ANWRWFHSTQ WLRGDPIKIL MPSLSPTMEE GNIVKWLKKE GEAVSAGDAL CEIETDKAVV TLDASDDGIL AKIVVEEGSK NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP VKKEHIPGTL RFRLSPAARN ILEKHSLDAS QGTATGPRGI FTKEDALKLV QLKQTGKITE SRPTPAPTAT PTAPSPLQAT AGPSYPRPVI PPVSTPGQPN AVGTFTEIPA SNIRRVIAKR LTESKSTVPH
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Réactivité croisée
- Humain, Souris, Rat
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Attributs du produit
- Polyclonal Antibodies
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Purification
- Affinity purification
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Immunogène
- Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).
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Isotype
- IgG
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Indications d'application
- WB,1:500 - 1:2000,IP,1:50 - 1:200
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Commentaires
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HIGH QUALITY
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Restrictions
- For Research Use only
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Format
- Liquid
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Buffer
- PBS with 0.02 % sodium azide,50 % glycerol, pH 7.3.
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Agent conservateur
- Sodium azide
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Précaution d'utilisation
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Stock
- -20 °C
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Stockage commentaire
- Store at -20°C. Avoid freeze / thaw cycles.
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- PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
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Autre désignation
- PDHX
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Sujet
- The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit, also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95 % of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.,PDHX,DLDBP,E3BP,OPDX,PDX1,proX,Cancer,Signal Transduction,Endocrine & Metabolism,Carbohydrate metabolism,Warburg Effect,PDHX
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Poids moléculaire
- 29 kDa/51 kDa/54 kDa
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ID gène
- 8050
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UniProt
- O00330
Antigène
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