製品名
Anti-α-Dystroglycan Antibody, clone IIH6C4, ascites fluid, clone IIH6C4, Upstate®
biological source
mouse
antibody form
ascites fluid
clone
IIH6C4, monoclonal
species reactivity
human, mouse, canine, rat, guinea pig, rabbit
manufacturer/tradename
Upstate®
technique(s)
immunofluorescence: suitable
immunohistochemistry: suitable
inhibition assay: suitable
western blot: suitable
isotype
IgM
NCBI accession no.
UniProt accession no.
shipped in
dry ice
target post-translational modification
unmodified
Gene Information
human ... DAG1(1605)
Analysis Note
Control
Rabbit skeletal muscle lysate.
Rabbit skeletal muscle lysate.
Routinely evaluated by western blot on rabbit skeletal muscle.
Western Blot Analysis:
A 1:1000-1:2000 dilution of this lot detected α-Dystroglycan/LARGE-glycan in rabbit skeletal muscle.
Note: The use of WGA purified protein results in significantly cleaner blots and immunoprecipitates.
Post-translational modification of dystroglycan causes band broadening.
Western Blot Analysis:
A 1:1000-1:2000 dilution of this lot detected α-Dystroglycan/LARGE-glycan in rabbit skeletal muscle.
Note: The use of WGA purified protein results in significantly cleaner blots and immunoprecipitates.
Post-translational modification of dystroglycan causes band broadening.
Application
Inhibition of Laminin Binding to Dystroglycan: An independent lab has shown, in a nitrocellulose overlay experiment, that this antibody inhibits binding of 125I-laminin to dystroglycan (Ervasti, J., et al. (1993).
Western Blot Analysis: A previous lot of this antibody was used on mouse muscle tissue lysate 3 months after shRNA induction, and in littermate controls (ctrl) and LARGE-null negative control (myd) (Goddeeris, M., et al. 2013, Nature).
Immunofluorescence: A previous lot of this antibody was used to detect α-Dystroglycan /LARGE-glycan in mouse muscle tissue (Goddeeris, M., et al. 2013, Nature).
Western Blot Analysis: A previous lot of this antibody was used on mouse muscle tissue lysate 3 months after shRNA induction, and in littermate controls (ctrl) and LARGE-null negative control (myd) (Goddeeris, M., et al. 2013, Nature).
Immunofluorescence: A previous lot of this antibody was used to detect α-Dystroglycan /LARGE-glycan in mouse muscle tissue (Goddeeris, M., et al. 2013, Nature).
Research Category
Metabolism
Metabolism
Research Sub Category
Muscle Physiology
Muscle Physiology
This Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.
Biochem/physiol Actions
This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
General description
156 kDa
Dystroglycans are essential elements of the neuromuscular junction (NMJ). The gene for dystroglycan is expressed as a precursor protein that is post translationally cleaved into a 156 kDa extracellular peripheral membrane protein called alpha dystroglycan and a 43 kDa transmembrane protein, beta dystroglycan. The latter protein contains a PPxY motif that promotes binding to WW domain containing proteins, such as utrophin and dystrophin. Phosphorylation at tyrosine 892 within the PPxY motif may regulate c Src interactions with beta dystroglycan, as well as inhibit interactions with WW domain proteins. In skeletal muscle, beta dystroglycan is normally localized to the plasma membrane, however phosphorylation of Tyr892 leads to localization of beta dystroglycan to endosomal compartments along with c Src. Thus, phosphorylation at Tyr892 may have important roles in altering the localization of beta dystroglycan during NMJ formation.
This product may be used for research purposes only. Diagnostic use of this product requires a license from the University of Iowa Research Foundation, 214 Technology Innovation Center, Iowa City, IA 52242
Immunogen
Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Physical form
Mouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol.
Liquid at -20ºC.
Liquid at -20ºC.
Unpurified
Preparation Note
Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.
Legal Information
UPSTATE is a registered trademark of Merck KGaA, Darmstadt, Germany
保管分類
12 - Non Combustible Liquids
wgk
WGK 2
flash_point_f
Not applicable
flash_point_c
Not applicable
試験成績書(COA)
製品のロット番号・バッチ番号を入力して、試験成績書(COA) を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
Mice Lacking Dystrophin or {alpha} Sarcoglycan Spontaneously Develop Embryonal Rhabdomyosarcoma with Cancer-Associated p53 Mutations and Alternatively Spliced or Mutant Mdm2 Transcripts.
Fernandez K, Serinagaoglu Y, Hammond S, Martin LT, Martin PT
The American Journal of Pathology null
A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin.
Ervasti, J M and Campbell, K P
The Journal of cell biology, 122, 809-823 (1993)
Susan Sparks et al.
BMC neurology, 7, 3-3 (2007-01-31)
Hereditary Inclusion Body Myopathy (HIBM) is an autosomal recessive, adult onset, non-inflammatory neuromuscular disorder with no effective treatment. The causative gene, GNE, codes for UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase, which catalyzes the first two reactions in the synthesis of sialic acid. Reduced
Francesca Sciandra et al.
BMC research notes, 10(1), 601-601 (2017-11-22)
Dystroglycan (DG) is an adhesion complex formed by two subunits, α-DG and β-DG. In skeletal muscle, DG is part of the dystrophin-glycoprotein complex that is crucial for sarcolemma stability and it is involved in a plethora of muscular dystrophy phenotypes.
B Wu et al.
Gene therapy, 21(9), 785-793 (2014-06-20)
Antisense therapy with both chemistries of phosphorodiamidate morpholino oligomers (PMOs) and 2'-O-methyl phosphorothioate has demonstrated the capability to induce dystrophin expression in Duchenne muscular dystrophy (DMD) patients in phase II-III clinical trials with benefit in muscle functions. However, potential of
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