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Invitrogen™ GAA Polyclonal Antibody
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Catalog No. PIPA579290
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100 μg
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PIPA579290 100 μg
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Catalog No. PIPA579290 Supplier Invitrogen™ Supplier No. PA579290
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Description

Rabbit Polyclonal Antibody

Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 μg/mL. Positive Control - WB: human A549 whole cell, human HepG2 whole cell, human HEK293 whole cell, human PC-3 whole cell. IHC: human liver cancer tissue, human prostatic cancer tissue.

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.
TRUSTED_SUSTAINABILITY

Specifications

Antigen GAA
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 500 μg/mL
Conjugate Unconjugated
Formulation PBS with 4mg trehalose and 0.05mg sodium azide
Gene Gaa
Gene Accession No. P10253
Gene Alias 70 kDa lysosomal alpha-glucosidase; 76 kDa lysosomal alpha-glucosidase; acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; acid maltase; Aglucosidase alfa; E430018M07Rik; Gaa; glucosidase alpha, acid; glucosidase, alpha, acid; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); LYAG; Lysosomal alpha-glucosidase
Gene Symbols Gaa
Host Species Rabbit
Immunogen A synthetic peptide corresponding to a sequence in the middle region of human GAA (494-527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR).
Purification Method Antigen affinity chromatography
Quantity 100 μg
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 2548
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Lyophilized
Isotype IgG
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