Anti-GAA antibody (100-200) [S6MR] (STJ11102966)

Name: Anti-GAA antibody (100-200) [S6MR] (STJ11102966)
SKU: STJ11102966
Availability: InStock

⇓ Datasheet ⇓ SDS

SPECIFICATIONS

ClonalityMonoclonal

HostRabbit

ConjugationUnconjugated

IsotypeIgG

STJ11102966

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Short Description	Rabbit monoclonal GAA (100-200) antibody for use in WB and ELISA in human samples. Datasheet included with dilution recommendations, and related reagents.
Applications	WB/ELISA
Host	Rabbit
Reactivity	Human
Note	STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.

Clonality	Monoclonal
Clone ID	S6MR
Isotype	IgG
Conjugation	Unconjugated
Concentration	Lot specific
Purification	Affinity purification
Dilution Range	WB:1:1000-1:6000 ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements.
Formulation	PBS with 0.02% Sodium Azide, 0.05% BSA, 50% Glycerol, pH 7.3.
Storage Instruction	Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles.

Gene Symbol	GAA
Gene ID	2548
Uniprot ID	LYAG_HUMAN
Immunogen Region	100-200
Immunogen Sequence	QEQCEARGCCYIPAKQGLQG AQMGQPWCFFPPSYPSYKLE NLSSSEMGYTATLTRTTPTF FPKDILTLRLDVMMETENRL HFTIKDPANRRYEVPLETPH V
Specificity	A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).

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