AGL Antibody (C-term) | MyBioSource MBS9201231 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

AGL Antibody (C-term)

catalog :

MBS9201231

quantity :

0.08 mL

price :

165 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, ELISA, immunocytochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

image

image 1 :

Anti-AGL Antibody (C-term) at 1:8000 dilution + human skeletal muscle lysate Lysates/proteins at 20 ug per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 175 kDa Blocking/Dilution buffer: 5% NFDM/TBST.

image 2 :

Western blot using anti-AGL (C-term) antibody (MBS9217121) at 1:1000 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.

image 3 :

Expression of myc-GS causes wild type but not the Ã„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 Ã„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.

product information

catalog number :

MBS9201231

products type :

Antibody

products full name :

AGL Antibody (C-term)

products short name :

[AGL]

products name syn :

[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; AGL; GDE]

other names :

[glycogen debranching enzyme isoform 1; Glycogen debranching enzyme; glycogen debranching enzyme; amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase; Glycogen debrancherIncluding the following 2 domains:4-alpha-glucanotransferase (EC:2.4.1.25)Alternative name(s):Oligo-1,4-1,4-glucantransferase]

products gene name :

[AGL]

other gene names :

[AGL; AGL; GDE; GDE; Amylo-1,6-glucosidase]

uniprot entry name :

GDE_HUMAN

clonality :

Polyclonal

isotype :

Rabbit Ig

host :

Rabbit

reactivity :

Human

sequence positions :

[1479-1510]

sequence length :

1,516

specificity :

This AGL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1479-1510 amino acids from the C-terminal region of human AGL.

purity :

Purified Rabbit Polyclonal Antibody (Pab)

form :

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.

concentration :

Vial Concentration: 2

storage stability :

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.

tested application :

Western Blot (WB), ELISA (EIA), Immunofluorescence (IF)

app notes :

WB~~1:1000

image1 heading :

Western Blot

image2 heading :

Western Blot (WB)

image3 heading :

Immunofluorescence (IF)

image4 heading :

Immunofluorescence (IF)

image4 description :

Confocal immunofluorescent analysis of AGL Antibody (C-term) with HepG2 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red).DAPI was used to stain the cell nuclear (blue).

other info1 :

Antigen Type: Synthetic Peptide

other info2 :

Antigen Source: HUMAN. Function: Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4- alpha-D-glucan4-alpha-D- glycosyltransferase and amylo-1,6-glucosidase in glycogen degradation. 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

products categories :

Cancer; Signal Transduction

products 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.

products references :

Horinishi, A., et al., J. Hum. Genet. 47(2):55-59 (2002). Bao, Y., et al., Genomics 38(2):155-165 (1996). Yang, B.Z., et al., J. Biol. Chem. 267(13):9294-9299 (1992). Yang-Feng, T.L., et al., Genomics 13(4):931-934 (1992). Bao, Y., et al., Gene 197 (1-2), 389-398 (1997).

ncbi gi num :

116734847

ncbi acc num :

NP_000019.2

ncbi gb acc num :

NM_000028.2

uniprot acc num :

P35573

ncbi mol weight :

174764

ncbi pathways :

2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178); 2-oxopentenoate Degradation Pathway (178)

ncbi 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, Jul 2008]

uniprot summary :

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; EC 2.4.1.25; Transferase; EC 3.2.1.33; Carbohydrate Metabolism - starch and sucrose; Hydrolase. Chromosomal Location of Human Ortholog: 1p21. Cellular Component: nucleoplasm; sarcoplasmic reticulum; cytoplasm; isoamylase complex; inclusion body; cytosol. Molecular Function: protein binding; glycogen debranching enzyme activity; 4-alpha-glucanotransferase activity; polyubiquitin binding; amylo-alpha-1,6-glucosidase activity; polysaccharide binding. Biological Process: glycogen biosynthetic process; glycogen catabolic process; carbohydrate metabolic process; response to glucocorticoid stimulus; glucose metabolic process; pathogenesis; response to nutrient. Disease: Glycogen Storage Disease Iii

size1 :

0.08 mL

price1 :

165 USD

size2 :

0.4 mL

price2 :

370

more info or order :

MyBioSource product webpage