GAA antibody (N-Term)

Catalog No. ABIN2781771

Product Details anti-GAA Antibody

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

Binding Specificity: N-Term

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)

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 dilutions should be determined experimentally by the investigator.

Comment:

Antigen size: 952 AA

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: Lot specific

Buffer: Liquid. 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: Avoid repeated freeze-thaw cycles.

Storage: -20 °C

Storage Comment: For short term use, store at 2-8°C 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 (GAA Products)

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. 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.
Alias Symbols: LYAG
Protein Interaction Partner: UBC, SUMO1, NEDD8, NUMBL, STAT2, HIVEP1, EP300, SYNCRIP, MTHFD1, ILF3, HNRNPK, CDH2, CALU, FBXO6, NCF1,
Protein Size: 952

Molecular Weight: 98 kDa

Gene ID: 2548

NCBI Accession: NM_000152, NP_000143

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process