SAB5702125

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

• Synonym(e): GAA, LYAG, glucosidase alpha, acid
• UNSPSC-Code: 12352203

Eigenschaften

• Biologische Quelle: rabbit
• Qualitätsniveau: 100
• Materialien: colorless
• Klon: 5L2L10, monoclonal
• Form: liquid
• Speziesreaktivität: rat, rat, human
• Konzentration: 0.8 mg/mL
• Methode(n): western blot: 1:500 - 1:2000
• Farbe: colorless
• Isotyp: IgG
• Immunogene Sequenz: QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
• UniProt-Hinterlegungsnummer: P10253
• Versandbedingung: wet ice
• Lagertemp.: −20°C
• Posttranslationale Modifikation Target: unmodified
• Angaben zum Gen: human ... GAA(2548)

Beschreibung

Allgemeine Beschreibung

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe′s disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).

PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

Anwendung

WB

Sicherheitshinweise

• Lagerklassenschlüssel: 10 - Combustible liquids
• WGK: WGK 1