GAA antibody (N-Term)

Catalog No. ABIN2781771

The Rabbit Polyclonal anti-GAA antibody (ABIN2781771) specifically detects GAA in WB and IHC. The antibody is reactive with Human, Mouse, Rat, Cow, Dog, Guinea Pig, Zebrafish (Danio rerio), Horse and Rabbit samples.

Quick Overview for GAA antibody (N-Term) (ABIN2781771)

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

Reactivity: Human, Mouse, Rat, Cow, Dog, Guinea Pig, Zebrafish (Danio rerio), Horse, Rabbit

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GAA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Product Details anti-GAA Antibody

Binding Specificity: N-Term

Purpose: GAA Antibody - N-terminal region

Sequence: FGVIVRRQLD GRVLLNTTVA PLFFADQFLQ LSTSLPSQYI TGLAEHLSPL

Predicted Reactivity: Cow: 100%, Dog: 100%, Guinea Pig: 100%, Horse: 93%, Human: 100%, Mouse: 100%, Rabbit: 79%, Rat: 100%, Zebrafish: 83%

Characteristics: This is a rabbit polyclonal antibody against GAA. It was validated on Western Blot using a cell lysate as a positive control.

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 AAP44226-100UG

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: 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. This gene encodes 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. Three transcript variants encoding the same protein have been found for this gene.

Gene Name: Glucosidase, alpha, acid

Alternative Symbols: LYAG

Protein Name: Lysosomal alpha-glucosidase

Molecular Weight: 98kDa

Gene ID: 2548

NCBI Accession: NP_000143

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process