コンテンツへスキップ
Merck

05-593

Anti-α-Dystroglycan Antibody, clone IIH6C4

ascites fluid, clone IIH6C4, Upstate®

別名:

Dystrophin-associated glycoprotein 1, dystroglycan 1, dystroglycan 1 (dystrophin-associated glycoprotein 1), dystrophin-associated glycoprotein-1, LARGE-glycan, Large glycan

ログインで組織・契約価格をご覧ください。

サイズを選択してください


この商品について

UNSPSC Code:
12352203
NACRES:
NA.41
eCl@ss:
32160702
テクニカルサービス
お困りのことがあれば、経験豊富なテクニカルサービスチームがお客様をサポートします。
お手伝いします
テクニカルサービス
お困りのことがあれば、経験豊富なテクニカルサービスチームがお客様をサポートします。
お手伝いします

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)

General description

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
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.

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.
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

Analysis Note

Control
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.

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).
Research Category
Metabolism
Research Sub Category
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.

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。

以前この製品を購入いただいたことがある場合

文書ライブラリで、最近購入した製品の文書を検索できます。

文書ライブラリにアクセスする

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
Katharine M Sharpe et al.
Journal of muscle research and cell motility, 34(5-6), 395-405 (2013-10-08)
Heart disease is a leading cause of death in patients with Duchenne muscular dystrophy (DMD). Patients with DMD lack the protein dystrophin, which is widely expressed in striated muscle. In skeletal muscle, the loss of dystrophin results in dramatically decreased
Membrane organization of the dystrophin-glycoprotein complex.
Ervasti, J M and Campbell, K P
Cell, 66, 1121-1131 (1991)
Jihee Kim et al.
Skeletal muscle, 6, 3-3 (2016-02-24)
The defective glycosylation of α-dystroglycan is associated with a group of muscular dystrophies that are collectively referred to as the secondary dystroglycanopathies. Mutations in the gene encoding fukutin-related protein (FKRP) are one of the most common causes of secondary dystroglycanopathy
Huaiyu Hu et al.
Genes, 7(12) (2016-12-06)
Patients with type II lissencephaly, a neuronal migration disorder with ectopic neurons, suffer from severe mental retardation, including learning deficits. There is no effective therapy to prevent or correct the formation of neuronal ectopia, which is presumed to cause cognitive

ライフサイエンス、有機合成、材料科学、クロマトグラフィー、分析など、あらゆる分野の研究に経験のあるメンバーがおります。.

製品に関するお問い合わせはこちら(テクニカルサービス)