Rabbit anti-Human GAA Monoclonal Antibody

The antibody against GAA was raised in Rabbit using a synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253) as the immunogen. The monoclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.

ADA-14230A

Number

Cat.No	ADA-14230A	Clonality	Monoclonal
Host Species	Rabbit	Target Name	GAA
Target Synonyms	LYAG; GAA	Form	Liquid
Species Reactivity	Human	Isotype	IgG
Storage Buffer	50% Glycerol, 0.05% BSA, PBS with 0.02% sodium azide, pH7.3.	Purification Method	Affinity purification
Positive Samples	293T, DU145, HepG2	Application	ELISA, WB

Cat.No

ADA-14230A

Clonality

Monoclonal

Host Species

Rabbit

Target Name

GAA

Target Synonyms

LYAG; GAA

Form

Liquid

Species Reactivity

Human

Isotype

IgG

Storage Buffer

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

Purification Method

Affinity purification

Positive Samples

293T, DU145, HepG2

Application

ELISA, WB

Immunogen Description	A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).	Target Species	Human
Immunogen Sequence	QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV	Uniprot ID	P10253

Immunogen Description

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

Target Species

Human

Immunogen Sequence

QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV

Uniprot ID

P10253

Background Information

Uniprot Id

P10253
Target Species

Human
Target Name

GAA
Target Full Name

Lysosomal alpha-glucosidase
Target Function

Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans.
Target Involvement

Glycogen storage disease 2 (GSD2)
Target Subcellular Location

Lysosome. Lysosome membrane.
Target Protein Families

Glycosyl hydrolase 31 family
Target Synonyms

70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid (Pompe disease glycogen storage disease type II); Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase
Target Background

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.

Rabbit anti-Human GAA Monoclonal Antibody

Specifications

Immunogen Information

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