Thermo Fisher Scientific GAA Monoclonal Antibody (3C6)

Applications

Tested Dilution

Publications

Western Blot (WB)

1-5 µg/mL

-

ELISA (ELISA)

0.3 ng/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG1, kappa

Class

Monoclonal

Type

Antibody

Clone

3C6

Immunogen

GAA (AAH40431, 851 a.a. approximately 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:GAA,uniProtId:P10253-1,ncbiNodeId:9606,antigenRange:851-952,antigenLength:952,antigenImageFileName:H00002548-M01_GAA_P10253-1_House_mouse.svg,antigenImageFileNamePDP:H00002548-M01_GAA_P10253-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

See Label

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.4

Contains

no preservative

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Sequence of this protein is as follows: GEARGELFWD DGESLEVLER GAYTQVIFLA RNNTIVNELV RVTSEGAGLQ LQKVTVLGVA TAPQQVLSNG VPVSNFTYSP DTKVLDICVS LLMGEQFLVS WC

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.