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GAA Antibody / Glucosidase alpha acid

GAA Antibody / Glucosidase alpha acid size: 0.1mg 406

Supplier NJS POLY
Price 406
Size 0.1mg
Contact us Ask Component cell markers and DNA bp. markers are genetic markerser genes or DNA sequenceswith selection audit
CategoryAntibody
Concentration2ml sterile DI water, 5mg/ml if reconstituted with 0
FormAntigen affinity purified
ConjugationUnconjugated
ClonePolyclonal antibody
Recognised antigenGAA / Glucosidase alpha acid
Host animalRabbit (Oryctolagus cuniculus)
ClonalityPolyclonal (rabbit origin)
Species reactivity Due to limited knowledge and inability to test the antibody against all known species, Rat , we cannot guarantee that no other cross reactivity can occur, Human (Homo sapiens)
Tested applications IHC-P, WB
Recommended dilutions5-1ug/ml, Immunohistochemistry (FFPE): 1-2ug/ml, Western blot: 0
NotesOptimal dilution of the GAA antibody should be determined by the researcher
Added buffer025% sodium azide, 5% BSA and 0, Lyophilized from 1X Phosphate Buffered Saline (PBS) with 2
Intented useThis GAA antibodyis to be used only for research purposes and not for diagnostics
Uniprot #P10253
PurityAntigen affinity
Description Alternative splicing results in multiple transcript variants, Defects in this gene are the cause of glycogen storage disease II, The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme, This gene encodes lysosomal alpha-glucosidase, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum, which is essential for the degradation of glycogen to glucose in lysosomes, Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene
ImmunogenAmino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody
Storage Avoid repeated freezing and thawing, Celcius or lower, Cycles of freezing and thawing can denaturate the peptide chains of the antibodies and reduce their sensitivity and/or change their affinity, Prepare aliqotes in such a manner so that freeze-thaw cycles are minimized, aliquot and store at -20 deg, the GAA antibody may be kept for up to one month refrigerated at +4 degrees C, After reconstitution, For long-term
Localization membranous, Cytoplasmic
PropertiesC, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by NJS poly they should be stored frozen at - 24°
Gene targetGAA / Glucosidase alpha acid
Short nameGAA Antibody / Glucosidase alpha acid
Technique antibodies against human proteins, antibodies for, Antibody
Alternative nameGAA (Antibody to) / Glucosidase a acid
Alternative techniqueantibodies
Identity 4065
Gene GAA
Long gene name acid , glucosidase alpha
Synonyms gene name acid , alpha, glucosidase
Synonyms name Pompe disease glycogen storage disease type II
Locus 17q25, 3
Discovery year 1986-01-01
Entrez gene record 2548
Havana BLAST/BLAT OTTHUMG00000177537
Locus Specific Databases GAA - Pompe disease (glycogen storage disease type II) LOVD - Leiden Open Variation Database LRG_673

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