Anti-PKLR antibody [AT1E3] ab119500 Product datasheet 1 Image Overview

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Product datasheet
Anti-PKLR antibody [AT1E3] ab119500
1 Image
Overview
Product name
Anti-PKLR antibody [AT1E3]
Description
Mouse monoclonal [AT1E3] to PKLR
Tested applications
WB, ELISA
Species reactivity
Reacts with: Human
Immunogen
Recombinant fragment, corresponding to amino acids 47-574 of Human PKLR, purified from E.
coli (NP_000289)
Positive control
293T cell lysate
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage buffer
pH: 7.40
Preservative: 0.1% Sodium azide
Constituent: 99% PBS
Purity
Protein G purified
Clonality
Monoclonal
Clone number
AT1E3
Isotype
IgG1
Light chain type
kappa
Applications
Our Abpromise guarantee covers the use of ab119500 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application
Abreviews
Notes
WB
1/500. Predicted molecular weight: 62 kDa.
ELISA
Use at an assay dependent dilution.
Target
1
Function
Plays a key role in glycolysis.
Pathway
Carbohydrate degradation; glycolysis; pyruvate from D-glyceraldehyde 3-phosphate: step 5/5.
Involvement in disease
Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:102900]; also
known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by
increase of red blood cell ATP.
Defects in PKLR are the cause of pyruvate kinase deficiency of red cells (PKRD) [MIM:266200].
A frequent cause of hereditary non-spherocytic hemolytic anemia. Clinically, pyruvate kinasedeficient patients suffer from a highly variable degree of chronic hemolysis, ranging from severe
neonatal jaundice and fatal anemia at birth, severe transfusion-dependent chronic hemolysis,
moderate hemolysis with exacerbation during infection, to a fully compensated hemolysis without
apparent anemia.
Sequence similarities
Belongs to the pyruvate kinase family.
Anti-PKLR antibody [AT1E3] images
Anti-PKLR antibody [AT1E3] (ab119500) at
1/500 dilution + 293T cell lysate at 40 µg
Secondary
goat anti-mouse HRP
developed using the ECL technique
Predicted band size : 62 kDa
Observed band size : 62 kDa
Western blot - Anti-PKLR antibody [AT1E3]
(ab119500)
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"
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Terms and conditions
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