GAA antibody (N-Term)

Catalog No. ABIN635720

Product Details anti-GAA Antibody

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

Binding Specificity: N-Term

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB)

Specificity: GAA antibody was raised against the N terminal of GAA

Purification: Affinity purified

Immunogen: GAA antibody was raised using the N terminal of GAA corresponding to a region with amino acids FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

Application Details

Application Notes: WB: 1 µg/mL
Optimal conditions should be determined by the investigator.

Comment:

GAA Blocking Peptide, catalog no. 33R-2917, is also available for use as a blocking control in assays to test for specificity of this GAA antibody

Restrictions: For Research Use only

Handling

Format: Lyophilized

Reconstitution: Lyophilized powder. Add distilled water for a 1 mg/mL concentration of GAA antibody in PBS

Concentration: Lot specific

Buffer: PBS

Handling Advice: Avoid repeated freeze/thaw cycles.
Dilute only prior to immediate use.

Storage: 4 °C/-20 °C

Storage Comment: Store at 2-8 °C for short periods. For longer periods of storage, store at -20 °C.

Target Details for GAA

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

Alternative Name: GAA (GAA Products)

Synonyms: LYAG antibody, E430018M07Rik antibody, glucosidase alpha, acid antibody, glucosidase, alpha, acid antibody, transmembrane and coiled-coil domain family 1 antibody, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antibody, GAA antibody, Gaa antibody, TMCC1 antibody, gaa antibody

Background: GAA is 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.

Molecular Weight: 98 kDa (MW of target protein)

Pathways: Cellular Glucan Metabolic Process