Anti-AGL monoclonal antibody (DCABH-10456) Made to order

Rabbit anti-Human AGL monoclonal antibody for FC, ICC/IF, IHC-P, WB

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Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Species Reactivity
Mouse, Rat, Human
Immunogen
A synthetic peptide of human AGL is used for rabbit immunization.
Conjugate
Unconjugated

Target


Alternative Names
AGL; amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase; amylo 1, 6 glucosidase, 4 alpha glucanotransferase; glycogen debranching enzyme; glycogen storage disease type III; glycogen debrancher
Entrez Gene ID
UniProt ID

Product Background


Gene summary
AGL (Amylo-Alpha-1, 6-Glucosidase, 4-Alpha-Glucanotransferase) is a Protein Coding gene. Diseases associated with AGL include glycogen storage disease iiia and glycogen storage disease due to glycogen debranching enzyme deficiency. Among its related pathways are Metabolism and Glycosaminoglycan metabolism. GO annotations related to this gene include carbohydrate binding and polysaccharide binding. This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described.
Antigen Description
This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described. Glycogen storage disease 3 (GSD3) [MIM:232400]: A metabolic disorder associated with an accumulation of abnormal glycogen with short outer chains. It is clinically characterized by hepatomegaly, hypoglycemia, short stature, and variable myopathy. Glycogen storage disease type 3 includes different forms: GSD type 3A patients lack glycogen debrancher enzyme activity in both liver and muscle, while GSD type 3B patients are enzyme-deficient in liver only. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the liver, by various hormones including insulin and glucagon, to maintain a homeostatic balance of blood-glucose levels. When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result. Together with phosphorylase, glycogen debranching enzymes function in glycogen breakdown and glucose mobilization. When phosphorylase has digested a glycogen branch down to four glucose residues, it will not remove further residues. Glycogen debranching enzymes assist phosphorylase, the primary enzyme involved in glycogen breakdown, mobilize glycogen stores. Phosphorylase can only cleave α-1,4- glycosidic bond between adjacent glucose molecules in glycogen but branches exist as α-1,6 linkages. When phosphorylase reaches four residues from a branching point it stops cleaving; because 1 in 10 residues is branched, cleavage by phosphorylase alone would not be sufficient in mobilizing glycogen stores. Before phosphorylase can resume catabolism, debranching enzymes perform two functions:.
Pathway
Glucose metabolism, organism-specific biosystem; Glycogen Metabolism, organism-specific biosystem; Glycogen breakdown (glycogenolysis), organism-specific biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of carbohydrates, organism-specific biosystem; Starch and sucrose metabolism, organism-specific biosystem.

Citations


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References


DENNIS, RD; BAUMEISTER, S; et al. CHROMATOGRAPHIC AND ANTIGENIC PROPERTIES OF ECHINOCOCCUS-GRANULOSUS HYDATID CYST-DERIVED GLYCOLIPIDS. PARASITE IMMUNOLOGY 15:669-681(1993).
Liu, X; Wu, D; et al. Optimization of the production of Aspergillus niger alpha-glucosidase expressed in Pichia pastoris. WORLD JOURNAL OF MICROBIOLOGY & BIOTECHNOLOGY 29:533-540(2013).

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