GAA Antibody-N-terminal region

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Product Specifications

CAS Number

9007-83-4

Gene Name

Glucosidase, alpha; acid

Gene Aliases

LYAG

Gene ID

2548

Swiss Prot

P10253

Accession Number

NP_000143

Reactivity

Human

Immunogen

The immunogen is a synthetic peptide directed towards the N terminal region of human GAA

Target

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.

Partner Proteins

DUOX1; TXNDC11

Clonality

Polyclonal

Type

Polyclonal Antibody

Sequence

RDLAPTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSW

Applications

Purification

Affinity purified

Assay Protocol

Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol

Concentration

0.5 mg/ml

Homology

Dog: 86%; Guinea Pig: 100%; Horse: 86%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%

Format

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Reconstitution

For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.

Molecular Weight

104 kDa

Protein Length

952

NCBI Gene Symbol

GAA

Host or Source

Rabbit

Protein Name

Lysosomal alpha-glucosidase

Gene Name URL

GAA

Nucleotide Accession Number

NM_000152.3

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