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Mouse Anti-LYAG Recombinant Antibody (CBYCL-582) (CBMAB-L0483-YC)

Provided herein is a Mouse monoclonal antibody against Human LYAG. The antibody can be used for immunoassay techniques, such as WB, ICC, IHC-P, IHC-Fr, ELISA.
See all LYAG antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYCL-582
Antibody Isotype
IgG
Application
WB, ICC, IHC-P, IHC-Fr, ELISA

Basic Information

Immunogen
Glucosidase Alpha, Acid
Specificity
Human
Antibody Isotype
IgG
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!]

Buffer
PBS, pH7.4, with 0.02% sodium azide, 50% glycerol
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
glucosidase alpha, acid
Introduction
LYAG is 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.
Entrez Gene ID
2548
UniProt ID
P10253
Alternative Names
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 contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Diaphragm contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Glucose metabolic process1 PublicationIC:BHF-UCL
Glycogen catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Heart morphogenesisIEA:Ensembl
Locomotory behaviorIEA:Ensembl
Lysosome organizationManual Assertion Based On ExperimentIMP:BHF-UCL
Maltose metabolic process1 PublicationIC:BHF-UCL
Muscle cell cellular homeostasisIEA:Ensembl
Neuromuscular process controlling balanceIEA:Ensembl
Neuromuscular process controlling postureIEA:Ensembl
Regulation of the force of heart contractionIEA:Ensembl
Sucrose metabolic process1 PublicationIC:BHF-UCL
Tissue developmentIEA:Ensembl
Vacuolar sequesteringManual Assertion Based On ExperimentIMP: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.

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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