Rabbit Anti-GAA Recombinant Antibody (
5E5) (V2LY-0725-LY1250)

Name: Rabbit Anti-GAA Recombinant Antibody (5E5)
SKU: V2LY-0725-LY1250
Rating: 5 (1 reviews)

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Tested Data

Rabbit Anti-GAA Recombinant Antibody (5E5) (V2LY-0725-LY1250) in IHC

IHC image of V2LY-0725-LY1250 diluted at 1:50 and staining in paraffin-embedded human placenta tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.15% DAB.

Rabbit Anti-GAA Recombinant Antibody (5E5) (V2LY-0725-LY1250) in IHC

IHC image of V2LY-0725-LY1250 diluted at 1:50 and staining in paraffin-embedded human breast cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.15% DAB.

Datasheet

SDS

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Basic Information

Host Animal

Rabbit

Clone

5E5

Application

ELISA, IHC

Immunogen

A synthesized peptide derived from Human GAA.

Host Species

Rabbit

Specificity

Human

Antibody Isotype

IgG

Clonality

Monoclonal Antibody

Application Notes

Application	Note
IHC	1:50-1:200

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS, glycerol

Preservative

Sodium azide

Concentration

Batch dependent

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

glucosidase alpha,acid

Entrez Gene ID

2548

UniProt ID

P10253

Function

Essential for the degradation of glycogen in lysosomes (PubMed:1856189, PubMed:7717400, PubMed:14695532, PubMed:18429042).

Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).

Biological Process

Cardiac muscle contraction Source: BHF-UCL
Diaphragm contraction Source: BHF-UCL
Glucose metabolic process Source: BHF-UCL
Glycogen catabolic process Source: UniProtKB
Heart morphogenesis Source: Ensembl
Locomotory behavior Source: Ensembl
Lysosome organization Source: BHF-UCL
Maltose metabolic process Source: BHF-UCL
Muscle cell cellular homeostasis Source: Ensembl
Neuromuscular process controlling balance Source: Ensembl
Neuromuscular process controlling posture Source: Ensembl
Regulation of the force of heart contraction Source: Ensembl
Sucrose metabolic process Source: BHF-UCL
Tissue development Source: Ensembl
Vacuolar sequestering Source: BHF-UCL

Cellular Location

Lysosome; Lysosome membrane

Involvement in disease

Glycogen storage disease 2 (GSD2):
A metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy.

PTM

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.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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