Mouse Anti-GAA Recombinant Antibody (CBLG1-418) (CBMAB-G1580-LY)

Name: Mouse Anti-GAA Recombinant Antibody (CBLG1-418)
SKU: CBMAB-G1580-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

CBLG1-418

Application

IHC-P, WB

Immunogen

purified acid alpha-glucosidase from human placenta

Specificity

Human

Antibody Isotype

IgG1

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format

Liquid

Buffer

50% glycerol

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

Introduction

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. [provided by RefSeq, Jan 2016]

Entrez Gene ID

2548

UniProt ID

P10253

Alternative Names

Glucosidase Alpha; Acid; Aglucosidase Alfa; Acid Maltase; EC 3.2.1.20; Glycogen Storage Disease Type II; Lysosomal Alpha-Glucosidase; Glucosidase; Alpha; Acid; Pompe Disease; LYAG;

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.

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