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Pyruvate Dehydrogenase E1 beta subunit (10A1) Monoclonal Antibody

Applications

  • WB
  • FCM
  • IHC-P
  • IF(IHC-P)
  • IF(ICC)

Reactivity

  • Human
  • Mouse
  • Rat
Overview
Catalog # bsm-54110R
Product Name Pyruvate Dehydrogenase E1 beta subunit (10A1) Monoclonal Antibody
Applications WB, FCM, IHC-P, IF(IHC-P), IF(ICC)
Reactivity Human, Mouse, Rat
Specifications
Conjugation Unconjugated
Host Rabbit
Source Recombinant protein within C-terminal human Pyruvate Dehydrogenase E1 beta subunit
Clonality Monoclonal
Clone # 10A1
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 1xTBS (pH7.4), 1%BSA, 40%Glycerol and 0.05% Sodium Azide.
Storage Condition Store at 4C for up to 2 weeks. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
Target
Gene ID 5162
Swiss Prot P11177
Synonyms Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; PDHE1-B; PDHB; PHE1B; PDHBD; PDHE1B.
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).
Application Dilution
WB 1:300-5000
FCM 1:20-100
IHC-P 1:200-400
IF(IHC-P) 1:50-200
IF(ICC) 1:50-200