Thermo Fisher Scientific GAA Recombinant Rabbit Monoclonal Antibody (ARC2392)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:1,000-1:6,000

-

ELISA (ELISA)

1 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

ARC2392

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253) if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:GAA,uniProtId:P10253-1,ncbiNodeId:9606,antigenRange:100-200,antigenLength:952,antigenImageFileName:MA5-37870_GAA_P10253-1_Rabbit.svg,antigenImageFileNamePDP:MA5-37870_GAA_P10253-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.8 mg/mL

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.3, with 50% glycerol, 0.05% BSA

Contains

0.02% sodium azide

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2897790

Product Specific Information

Positive Samples: 293T, HepG2, DU145

Immunogen sequence: QEQCEARGCC YIPAKQGLQG AQMGQPWCFF PPSYPSYKLE NLSSSEMGYT ATLTRTTPTF FPKDILTLRL DVMMETENRL HFTIKDPANR RYEVPLETPH V

Target Information

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.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.