HPA000962
Anti-PYGL antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sinonimo/i:
Anti-Glycogen phosphorylase, liver form antibody produced in rabbit
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About This Item
Coniugato:
unconjugated
application:
IF
IHC
IHC
Clone:
polyclonal
Reattività contro le specie:
human
citations:
9
tecniche:
immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:500-1:1000
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:500-1:1000
Prodotti consigliati
Origine biologica
rabbit
Livello qualitativo
Coniugato
unconjugated
Forma dell’anticorpo
affinity isolated antibody
Tipo di anticorpo
primary antibodies
Clone
polyclonal
Nome Commerciale
Prestige Antibodies® Powered by Atlas Antibodies
Stato
buffered aqueous glycerol solution
Reattività contro le specie
human
Convalida avanzata
independent
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
tecniche
immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:500-1:1000
Sequenza immunogenica
MRIDDVAALDKKGYEAKEYYEALPELKLVIDQIDNGFFSPKQPDLFKDIINMLFYHDRFKVFADYEAYVKCQDKVSQLYMNPKAWNTMVLKNIAASGKFSSDRTIKEYAQNIWNVEPSD
N° accesso UniProt
Condizioni di spedizione
wet ice
Temperatura di conservazione
−20°C
modifica post-traduzionali bersaglio
unmodified
Informazioni sul gene
human ... PYGL(5836)
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Descrizione generale
The gene PYGL (phosphorylase, glycogen, liver) is mapped to human chromosome 14q21-q22.
Immunogeno
Glycogen phosphorylase, liver form recombinant protein epitope signature tag (PrEST)
Applicazioni
Anti-PYGL antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Western Blotting (1 paper)
Western Blotting (1 paper)
Azioni biochim/fisiol
PYGL is also known as GSD6 (i.e. Glycogen storage disease type VI (GSD VI)). Glycogen storage disease (GSD) type VI is a disorder of glycogenolysis that is caused by deficiency of hepatic glycogen phosphorylase resulting in a reduced ability to mobilize glucose from glycogen. It is characterized in the untreated child by hepatomegaly, growth retardation, ketotic hypoglycemia after an overnight fast, and mild hypoglycemia after prolonged fasting (e.g., during an illness). It is usually a relatively mild disorder seen in infancy and childhood. The GSD6 disorder is inherited in an autosomal recessive manner.
Caratteristiche e vantaggi
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
Linkage
Corresponding Antigen APREST70378
Stato fisico
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Note legali
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Esclusione di responsabilità
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Codice della classe di stoccaggio
10 - Combustible liquids
Classe di pericolosità dell'acqua (WGK)
WGK 1
Punto d’infiammabilità (°F)
Not applicable
Punto d’infiammabilità (°C)
Not applicable
Dispositivi di protezione individuale
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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J Hendrickx et al.
Human genetics, 97(5), 551-556 (1996-05-01)
Several types of glycogen storage disease attributable to a deficiency of phosphorylase or phosphorylase kinase have been described. These diseases have been divided according to clinical symptoms, mode of inheritance, and affected tissue. However, this classification is questionable, as the
M Johanns et al.
Nature communications, 7, 10856-10856 (2016-03-10)
Biguanides such as metformin have previously been shown to antagonize hepatic glucagon-stimulated cyclic AMP (cAMP) signalling independently of AMP-activated protein kinase (AMPK) via direct inhibition of adenylate cyclase by AMP. Here we show that incubation of hepatocytes with the small-molecule
Expression of glycogen phosphorylase isoforms in cultured muscle from patients with McArdle's disease carrying the p.R771PfsX33 PYGM mutation.
Nogales-Gadea, G., et al.
PLoS ONE, 5(10) (2010)
Margaret P Adam et al.
GeneReviews(?), 2009 Apr 23 (Updated 2011 May 17) (2011-05-17)
Glycogen storage disease type VI (GSD VI), a disorder of glycogenolysis caused by deficiency of hepatic glycogen phosphorylase, is characterized in the untreated child by hepatomegaly, growth retardation, ketotic hypoglycemia after an overnight fast, and mild hypoglycemia after prolonged fasting
Gisela Nogales-Gadea et al.
PloS one, 5(10), doi:10-doi:10 (2010-10-20)
Mutations in the PYGM gene encoding skeletal muscle glycogen phosphorylase (GP) cause a metabolic disorder known as McArdle's disease. Previous studies in muscle biopsies and cultured muscle cells from McArdle patients have shown that PYGM mutations abolish GP activity in
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