GAA Antibody - N-terminal regio : FITC (ARP44226 P050-FITC)

Glucosidase, alpha; acid

LYAG

2548

P10253

NP_000143

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Zebrafish

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

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.

UBC; SUMO1; NEDD8; NUMBL; STAT2; HIVEP1; EP300; SYNCRIP; MTHFD1; ILF3; HNRNPK; CDH2; CALU; FBXO6; NCF1

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

IHC, WB

Affinity Purified

GAA is supported by BioGPS gene expression data to be expressed in MCF7

0.5 mg/ml

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

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

98kDa

Wan, L., (er) J. Neurol. (2008) In press

Wet Ice

952

GAA

GAA

Rabbit

Lysosomal alpha-glucosidase

NM_000152

FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL