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Mouse Anti-GAA Recombinant Antibody (2G7) (CBMAB-BR183LY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
2G7
Antibody Isotype
IgG2B
Application
FC: 1-3 μg/1x10 cells, IF: 1:1000 dilution, IHC-P: 0.5-1 μg/ml, ICC: 1:200 dilution, WB: 0.1-0.5 μg/ml

Basic Information

Immunogen
A synthetic peptide corresponding to a sequence at the N-terminus of human Synaptophysin(1-26aa MLLLADMDVVNQLVAGGQFRVVKEPL), identical to the related mouse and rat sequences
Specificity
Human
Antibody Isotype
IgG2B
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
Lyophilized
Preservative
0.05 mg sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

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
Lysosomal alpha-glucosidase;3.2.1.20
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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For research use only. Not intended for any clinical use.

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