Rabbit anti-Human AGL Polyclonal Antibody | anti-AGL antibody

AGL Antibody (N-term)

Cat. Num. AAA9217121

• Gene Names: AGL; GDE
• Reactivity: Human
• Applications: Western Blot, Immunofluorescence, ELISA
• Synonyms: AGL; Polyclonal Antibody; AGL Antibody (N-term); Glycogen debranching enzyme; Glycogen debrancher; 4-alpha-glucanotransferase; Oligo-1;4-1;4-glucantransferase; Amylo-alpha-1;6-glucosidase; Amylo-1;6-glucosidase; Dextrin 6-alpha-D-glucosidase; GDE; anti-AGL antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Ig
• Specificity: This AGL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the N-terminal region of human AGL.
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
• Sequence Length: 1532

• Applicable Applications for anti-AGL antibody: Western Blot (WB), Immunofluorescence (IF), ELISA (EIA)
• Application Notes: WB:~~1:1000; IF:~~1:10~50
• Subtitle: Purified Rabbit Polyclonal Antibody (Pab)
• Cellular Location: Cytoplasm. Note=Under glycogenolytic conditions localizes to the nucleus.
• Tissue Location: Liver, kidney and lymphoblastoid cells express predominantly isoform 1; whereas muscle and heart express not only isoform 1, but also muscle-specific isoform mRNAs (isoforms 2, 3 and 4). Isoforms 5 and 6 are present in both liver and muscle.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 2 weeks. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Anti-AGL Antibody (M15) at 1:1000 dilution + human skeletal muscle lysateLysates/proteins at 20 ug per lane. SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 175 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)

Immunofluorescence (IF)

(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.)

Related Product Information for anti-AGL antibody

Description: AGL is a 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 the AGL 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.

Background: Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4- alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6- glucosidase in glycogen degradation.

Product Categories/Family for anti-AGL antibody

Primary Antibodies; Cancer; Signal Transduction

References

Horinishi, A., et al., J. Hum. Genet. 47 (2):55-59 (2002). Shen, J., et al., Hum. Mutat. 9 (1):37-40 (1997). Bao, Y., et al., Genomics 38 (2):155-165 (1996). Shen, J., et al., J. Clin. Invest. 98 (2):352-357 (1996). Yang, B.Z., et al., J. Biol. Chem. 267 (13):9294-9299 (1992).

NCBI and Uniprot Product Information

• NCBI GI #: 116734847
• NCBI GeneID: 178
• NCBI Accession #: NP_000019.2
• NCBI GenBank Nucleotide #: NM_000028.2
• UniProt Accession #: P35573; P78354; P78544; Q59H92; Q6AZ90; Q9UF08; A6NCX7; A6NEK2; D3DT51
• Molecular Weight: 174764 Da
• NCBI Official Full Name: glycogen debranching enzyme isoform 1
• NCBI Official Synonym Full Names: amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase
• NCBI Official Symbol: AGL
• NCBI Official Synonym Symbols: GDE
• NCBI Protein Information: glycogen debranching enzyme
• UniProt Protein Name: Glycogen debranching enzyme
• UniProt Gene Name: AGL
• UniProt Synonym Gene Names: GDE; Amylo-1,6-glucosidase
• UniProt Entry Name: GDE_HUMAN

NCBI 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. [provided by RefSeq, Jul 2008]

Uniprot Description

GDE: Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4- alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6- glucosidase in glycogen degradation. Defects in AGL are the cause of glycogen storage disease type 3 (GSD3); also known as Forbes disease. GSD3 is a metabolic disorder associated with an accumulation of abnormal glycogen with short outer chains. Three GSD3 types are recognized: 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. In rare cases, selective loss of only 1 of the 2 debranching activities, glucosidase or transferase, results in GSD type 3C or type 3D, respectively. GSD3 is clinically characterized by hepatomegaly, hypoglycemia, short stature, and variable myopathy. Belongs to the glycogen debranching enzyme family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Ubiquitin conjugating system; Hydrolase; EC 3.2.1.33; Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.25; Transferase

Chromosomal Location of Human Ortholog: 1p21

Cellular Component: cytoplasm; cytosol; inclusion body; isoamylase complex; nucleus; sarcoplasmic reticulum

Molecular Function: 4-alpha-glucanotransferase activity; amylo-alpha-1,6-glucosidase activity; glycogen debranching enzyme activity; polysaccharide binding; polyubiquitin binding; protein binding

Biological Process: glycogen biosynthetic process; glycogen catabolic process; response to glucocorticoid stimulus; response to nutrient

Disease: Glycogen Storage Disease Iii