GAA Antilichaam: Biotine (ARP44227 P050-Biotine)

Glucosidase, alpha; acid

LYAG

2548

P10253

NP_000143

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

The immunogen is a synthetic peptide directed towards the following sequence APTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRST

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

Biotin

Polyclonal Antibody

Affinity Purified

0.5 mg/ml

Cow: 92%; Dog: 100%; Guinea Pig: 100%; Horse: 93%; Human: 100%; Mouse: 100%; Rat: 100%

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.

105 kDa

Wet Ice

952

GAA

GAA

Rabbit

Lysosomal alpha-glucosidase

NM_000152

APTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRST