GAA antibody (N-Term)

Catalog No. ABIN8101555

This Rabbit Polyclonal antibody specifically detects GAA in WB. It exhibits reactivity toward Human.

Quick Overview for GAA antibody (N-Term) (ABIN8101555)

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB)

Product Details anti-GAA Antibody

Binding Specificity: N-Term

Purpose: GAA Antibody - N-terminal region

Sequence: RDLAPTPGAN LYGSHPFYLA LEDGGSAHGV FLLNSNAMDV VLQPSPALSW

Predicted Reactivity: Dog: 86%, Guinea Pig: 100%, Horse: 86%, Human: 100%, Mouse: 100%, Rabbit: 100%, Rat: 100%

Purification: Affinity purified

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

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Comment:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP44227-100UG

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.5 mg/mL

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

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: prevent freeze-thaw cycles

Storage: 4 °C,-20 °C

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

Target Details for GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Alternative Name: GAA

Background:

Background Information: 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.

Gene Name: glucosidase, alpha, acid

Alternative Symbols: LYAG

Protein Name: lysosomal alpha-glucosidase

Molecular Weight: 104 kDa

Gene ID: 2548

NCBI Accession: NP_000143

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process