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Anti-PDHB Antibody (9C498) is a Rabbit antibody targeting PDHB. Anti-PDHB Antibody (9C498) can be used in WB,IHC,IP,FCM.
Pack Size | Price | Availability | Quantity |
---|---|---|---|
50 μL | $296 | 7-10 days | |
100 μL | $496 | 7-10 days |
Description | Anti-PDHB Antibody (9C498) is a Rabbit antibody targeting PDHB. Anti-PDHB Antibody (9C498) can be used in WB,IHC,IP,FCM. |
Ig Type | IgG |
Reactivity | Human,Mouse,Rat |
Application | |
Recommended Dose | WB: 1:500-2000; IHC: 1:50-200; FCM: 1:50-100 |
Antibody Type | Monoclonal |
Host Species | Rabbit |
Construction | Recombinant Antibody |
Purification | ProA affinity purified |
Appearance | Liquid |
Formulation | 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide. |
Research Background | The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO. It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency). PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy). |
Conjucates | Unconjugated |
Immunogen | Recombinant Protein |
Uniprot ID |
Stability & Storage | Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
Transport | Shipping with blue ice. |
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