Anti-GAA antibody (350-450) (STJ29987)

SPECIFICATIONS
ClonalityPolyclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
STJ29987
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General Information

Short DescriptionRabbit polyclonal GAA (350-450) antibody for use in WB, IF, ICC and ELISA in human, mouse and rat samples. Datasheet included with dilution recommendations, and related reagents.
ApplicationsWB/IF/ICC/ELISA
HostRabbit
ReactivityHuman/Mouse/Rat
NoteSTRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.

Product Properties

ClonalityPolyclonal
IsotypeIgG
ConjugationUnconjugated
ConcentrationLot specific
PurificationAffinity purification
Dilution RangeWB:1:500-1:1000
IF/ICC:1:50-1:200
ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements.
FormulationPBS with 0.05% Proclin300, 50% Glycerol, pH7.3.
Storage InstructionStore at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles.

Target Information

Gene SymbolGAA
Gene ID2548
Uniprot IDLYAG_HUMAN
Immunogen Region350-450
Immunogen SequenceVVQQYLDVVGYPFMPPYWGL GFHLCRWGYSSTAITRQVVE NMTRAHFPLDVQWNDLDYMD SRRDFTFNKDGFRDFPAMVQ ELHQGGRRYMMIVDPAISSS G
SpecificityA synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).

Additional Info

Post Translational Modifications The different forms of acid glucosidase are obtained by proteolytic processing. Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.
Function Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans.
Protein Name Lysosomal Alpha-Glucosidase
Acid Maltase
Aglucosidase Alfa Cleaved Into - 76 Kda Lysosomal Alpha-Glucosidase - 70 Kda Lysosomal Alpha-Glucosidase
Database Links Reactome: R-HSA-5357609
Reactome: R-HSA-6798695
Reactome: R-HSA-70221
Cellular Localisation Lysosome
Lysosome Membrane
Alternative Antibody Names Anti-Lysosomal Alpha-Glucosidase antibody
Anti-Acid Maltase antibody
Anti-Aglucosidase Alfa Cleaved Into - 76 Kda Lysosomal Alpha-Glucosidase - 70 Kda Lysosomal Alpha-Glucosidase antibody
Anti-GAA antibody

Information sourced from Uniprot.org

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