Anti-GAA Antibody Picoband® (Dylight550 Conjugate)

Boster Bio Anti-GAA Antibody Picoband® catalog # A01548. Tested in IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

Synonyms

Lysosomal alpha-glucosidase;3.2.1.20;Acid maltase;Aglucosidase alfa;76 kDa lysosomal alpha-glucosidase;70 kDa lysosomal alpha-glucosidase;GAA;

Gene Name

Lysosomal alpha-glucosidase

UniProt

P10253

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Clonality

Polyclonal

Tissue Specificity

Most abundant in heart, brain, liver, skeletal muscle and testis but absent in thymus and peripheral blood leukocytes.

Applications

IHC,WB

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Function

Essential for the degradation of glygogen to glucose in lysosomes.

Storage Conditions

Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.