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Merck
모든 사진(4)

주요 문서

05-593

Sigma-Aldrich

Anti-α-Dystroglycan Antibody, clone IIH6C4

ascites fluid, clone IIH6C4, Upstate®

동의어(들):

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

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About This Item

UNSPSC 코드:
12352203
eCl@ss:
32160702
NACRES:
NA.41
클론:
IIH6C4, monoclonal
application:
IF
IHC
WB
inhibition assay
종 반응성:
human, mouse, canine, rat, guinea pig, rabbit
기술:
immunofluorescence: suitable
immunohistochemistry: suitable
inhibition assay: suitable
western blot: suitable
citations:
96

생물학적 소스

mouse

항체 형태

ascites fluid

클론

IIH6C4, monoclonal

종 반응성

human, mouse, canine, rat, guinea pig, rabbit

제조업체/상표

Upstate®

기술

immunofluorescence: suitable
immunohistochemistry: suitable
inhibition assay: suitable
western blot: suitable

동형

IgM

NCBI 수납 번호

UniProt 수납 번호

배송 상태

dry ice

타겟 번역 후 변형

unmodified

유전자 정보

human ... DAG1(1605)

일반 설명

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

특이성

This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.

면역원

Rabbit skeletal muscle membrane preparation. Clone IIH6C4.

애플리케이션

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.

품질

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.

표적 설명

156 kDa

물리적 형태

Mouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol.
Liquid at -20ºC.
Unpurified

저장 및 안정성

Stable for 1 year at -20°C from date of receipt. For maximum recovery of product, centrifuge the vial prior to removing the cap.

분석 메모

Control
Rabbit skeletal muscle lysate.

기타 정보

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

법적 정보

UPSTATE is a registered trademark of Merck KGaA, Darmstadt, Germany

면책조항

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.

Storage Class Code

12 - Non Combustible Liquids

WGK

WGK 2

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable


시험 성적서(COA)

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문서 라이브러리 방문

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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