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AGL Antibody (Center)

Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - AGL Antibody (Center) AP2402a
    Western blot analysis of hAGL-C371 (Cat. #AP2402a) in K562 cell line lysates (35ug/lane). AGL (arrow) was detected using the purified Pab.
  • 3 - AGL Antibody (Center) AP2402a
    Confocal immunofluorescent analysis of AGL Antibody (Center)(Cat#AP2402a) with HepG2 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).
  • 产品详情
  • 文献引用 : 6
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Product info
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IF, E
Primary Accession P35573
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit Ig
Additional info
Gene ID 178
Other Names 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
Target/Specificity This AGL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 357-387 amino acids from the Central region of human AGL.
Dilution WB~~1:1000
IF~~1:10~50
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsAGL Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name AGL
Synonyms GDE
Function 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.
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
Research Areas
Loss of glycogen debranching enzyme AGL drives bladder tumor growth via induction of hyaluronic acid synthesis.
Author : Guin S1, Ru Y2, Agarwal N3, Ritterson Lew C4, Owens C2, Comi GP5, Theodorescu D6.
Clin Cancer Res. 2015 Oct 21. pii: clincanres.1706.2015. [Epub ahead of print]
26490312
Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice.
Author : Irimia JM1, Tagliabracci VS2, Meyer CM1, Segvich DM1, DePaoli-Roach AA1, Roach PJ3.
J Biol Chem. 2015 Jul 27. pii: jbc.M115.673897. [Epub ahead of print]
26216881
Genetic depletion of the malin E3 ubiquitin ligase in mice leads to lafora bodies and the accumulation of insoluble laforin.
Author : DePaoli-Roach AA1, Tagliabracci VS, Segvich DM, Meyer CM, Irimia JM, Roach PJ.
J Biol Chem. 2010 Aug 13;285(33):25372-81. doi: 10.1074/jbc.M110.148668. Epub 2010 Jun 10.
20538597
Fast-twitch sarcomeric and glycolytic enzyme protein loss in inclusion body myositis.
Author : Parker KC, Kong SW, Walsh RJ; Bch, Salajegheh M, Moghadaszadeh B, Amato AA, Nazareno R, Lin YY, Krastins B, Sarracino DA, Beggs AH, Pinkus JL, Greenberg SA.
Muscle Nerve. 2009 Jun;39(6):739-53. doi: 10.1002/mus.21230.
19291799
Abnormal metabolism of glycogen phosphate as a cause for Lafora disease.
Author : Tagliabracci VS, Girard JM, Segvich D, Meyer C, Turnbull J, Zhao X, Minassian BA, Depaoli-Roach AA, Roach PJ.
J Biol Chem. 2008 Dec 5;283(49):33816-25. doi: 10.1074/jbc.M807428200. Epub 2008 Oct 13.
18852261
A role for AGL ubiquitination in the glycogen storage disorders of Lafora and Cori\'s disease.
Author : Cheng A1, Zhang M, Gentry MS, Worby CA, Dixon JE, Saltiel AR.
Genes Dev. 2007 Oct 1;21(19):2399-409.
17908927

BACKGROUND

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.

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).

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