Anti-GAA Antibody Picoband® (monoclonal, 2G7)-iFluor647 Conjugate

About this product

Boster Bio Anti-GAA Antibody Picoband® (monoclonal, 2G7) catalog # M01548. Tested in IF, IHC, ICC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Specifications

Catalog number

M01548-iFluor647

Size / count

100 µg

Dry ice

No

Hazardous

No

Discontinued

Yes

Background

Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

Synonyms

Lysosomal alpha-glucosidase;3.2.1.20

Gene Name

glucosidase alpha,acid

Gene ID

2548

UniProt

P10253

Host

Mouse

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

Clonality

Monoclonal

Clone

Clone: 2G7

Tissue Specificity

Expressed on platelets and leukocytes and is primarily concentrated at the borders between endothelial cells. Expressed in human umbilical vein endothelial cells (HUVECs) (at protein level). Expressed on neutrophils (at protein level). Isoform Long predominates in all tissues examined. Isoform Delta12 is detected only in trachea. Isoform Delta14-15 is only detected in lung. Isoform Delta14 is detected in all tissues examined with the strongest expression in heart. Isoform Delta15 is expressed in brain, testis, ovary, cell surface of platelets, human umbilical vein endothelial cells (HUVECs), Jurkat T-cell leukemia, human erythroleukemia (HEL) and U-937 histiocytic lymphoma cell lines (at protein level).

Applications

IF,IHC,ICC,WB

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500μg/ml.

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.

Components

Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

References & Citations

1. "Entrez Gene: GAA glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)". 2. Donald J. Voet; Judith G. Voet; Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538. 3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61–9.

Storage Conditions

Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Observed Molecular Weight

110 kDa, 95 kDa, 76 kDa

Fragment

Mouse IgG2b

Specificity

No cross reactivity with other proteins.

Applications Notes

Western blot, 0.1-0.5μg/ml, Human<br> Immunohistochemistry (Paraffin-embedded Section), 0.5-1μg/ml, Human<br> Immunocytochemistry/Immunofluorescence, 2μg/ml, Human<br>

Subcellular Location

Lysosome

Protein Name

Lysosomal alpha-glucosidase