AGL polyclonal antibody
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Specification
Product Description
Rabbit polyclonal antibody raised against synthetic peptide of AGL.
Immunogen
A synthetic peptide (conjugated with KLH) corresponding to amino acids 1479-1510 at the C-terminus of human AGL.
Host
Rabbit
Reactivity
Human
Form
Liquid
Purification
Protein A purification
Recommend Usage
ELISA
Immunofluorescence (1:10-50)
Western Blot (1:8000)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS (0.09% sodium azide)
Storage Instruction
Store at 4°C. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Tissue lysate)
Western blot using AGL polyclonal antibody (Cat # PAB4298) at 1 : 500 dilution . A total of 20 ug of lysates was loaded for each tissue . Data courtesy of Dr . Alan Cheng, Department of Internal Medicine, Life Sciences Institute, University of Michigan Medical Center, Ann Arbor, Michigan .Immunofluorescence
Expression of myc-GS causes wild type but not the CBD mutant of AGL to aggregate around the PAS-stain-positive inclusions. HepG2 cells were transfected with either HA-tagged wild-type AGL (HA-AGL) or HA-AGL CBD. Cells were fixed in formalin and processed for IF using anti-HA (green) and anti-myc (red) antibodies. White arrows indicate colocalization of HA-AGL and myc-GS.Enzyme-linked Immunoabsorbent Assay
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Gene Info — AGL
Entrez GeneID
178Protein Accession#
NP_000019;P35573Gene Name
AGL
Gene Alias
GDE
Gene Description
amylo-1, 6-glucosidase, 4-alpha-glucanotransferase
Gene Ontology
HyperlinkGene Summary
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. [provided by RefSeq
Other Designations
OTTHUMP00000012500|OTTHUMP00000012501|OTTHUMP00000012502|OTTHUMP00000012503|OTTHUMP00000012504|OTTHUMP00000012505|glycogen debranching enzyme
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Interactome
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Pathway
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Disease
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Publication Reference
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A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease.
Cheng A, Zhang M, Gentry MS, Worby CA, Dixon JE, Saltiel AR.
Genes & Development 2007 Oct; 21(19):2399.
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A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori's disease.
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