Anti-a-Dystroglycan, clone IIH6C4

Species Reactivity	Key Applications	Host	Format	Antibody Type
H, R, M, Rb, Ca, Gp	WB, Inhibition, IHC, IF	M	Ascites	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

H, R, M, Rb, Ca, Gp

WB, Inhibition, IHC, IF

Ascites

Monoclonal Antibody

Description
Catalogue Number	05-593
Brand Family	Upstate
Trade Name	Upstate
Description	Anti-α-Dystroglycan Antibody, clone IIH6C4
Overview	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
Alternate Names	Dystrophin-associated glycoprotein 1 dystroglycan 1 dystroglycan 1 (dystrophin-associated glycoprotein 1) dystrophin-associated glycoprotein-1 LARGE-glycan Large glycan
Background Information	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.

Description

Catalogue Number

05-593

Brand Family

Upstate

Trade Name

Upstate

Description

Anti-α-Dystroglycan Antibody, clone IIH6C4

Overview

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

Alternate Names

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

Background Information

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.

Product Information
Format	Ascites
Control	Rabbit skeletal muscle lysate.
Presentation	Mouse ascites, in PBS containing 0.05% sodium azide and 30% glycerol. Liquid at -20ºC.

Product Information

Format

Ascites

Control

Rabbit skeletal muscle lysate.

Presentation

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

Applications
Application	This Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.
Key Applications	Western Blotting Inhibition Immunohistochemistry Immunofluorescence
Application Notes	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).

Applications

Application

This Anti-α-Dystroglycan Antibody, clone IIH6C4 is validated for use in IH, FUNC, WB for the detection of α-Dystroglycan.

Key Applications

Western Blotting
Inhibition
Immunohistochemistry
Immunofluorescence

Application Notes

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).

Biological Information
Immunogen	Rabbit skeletal muscle membrane preparation. Clone IIH6C4.
Clone	IIH6C4
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Mouse
Specificity	This antibody recognizes α-Dystroglycan/LARGE-glycan, Mr 156 kDa.
Isotype	IgM
Species Reactivity	Human Rat Mouse Rabbit Canine Guinea Pig
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_004393
Entrez Gene Summary	Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Dystroglycan 1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic reduction of dystroglycan 1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.
Gene Symbol	DAG1 A3a DAG beta-dystroglycan alpha-dystroglycan AGRNR 156DAG
Purification Method	Unpurified
UniProt Number	Q14118
UniProt Summary	FUNCTION: SwissProt: Q14118 # Forms part of the dystrophin-associated protein complex (DAPC) which may link the cytoskeleton to the extracellular matrix. Alpha-dystroglycan functions as a laminin receptor. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells. SIZE: 895 amino acids; 97581 Da SUBUNIT: Interacts with SGCD (By similarity). Interacts with AGR2 and AGR3. SUBCELLULAR LOCATION: Alpha-dystroglycan: Secreted, extracellular space. & Beta-dystroglycan: Cell membrane; Single- pass type I membrane protein. TISSUE SPECIFICITY: Expressed in a variety of fetal and adult tissues.
Molecular Weight	156 kDa

Biological Information

Immunogen

Rabbit skeletal muscle membrane preparation. Clone IIH6C4.

Clone

IIH6C4

Concentration

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

Host

Mouse

Specificity

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

Isotype

IgM

Species Reactivity

Human
Rat
Mouse
Rabbit
Canine
Guinea Pig

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NM_004393

Entrez Gene Summary

Dystroglycan is a laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. Dystroglycan 1 is a candidate gene for the site of the mutation in autosomal recessive muscular dystrophies. The dramatic reduction of dystroglycan 1 in Duchenne muscular dystrophy leads to a loss of linkage between the sarcolemma and extracellular matrix, rendering muscle fibers more susceptible to necrosis. Dystroglycan also functions as dual receptor for agrin and laminin-2 in the Schwann cell membrane. The muscle and nonmuscle isoforms of dystroglycan differ by carbohydrate moieties but not protein sequence.

Gene Symbol

DAG1
A3a
DAG
beta-dystroglycan
alpha-dystroglycan
AGRNR
156DAG

Purification Method

Unpurified

UniProt Number

Q14118

UniProt Summary

FUNCTION: SwissProt: Q14118 # Forms part of the dystrophin-associated protein complex (DAPC) which may link the cytoskeleton to the extracellular matrix. Alpha-dystroglycan functions as a laminin receptor. Binds to several types of arenaviruses. Is a target for the entry of Mycobacterium leprae into peripheral nerve Schwann cells.
SIZE: 895 amino acids; 97581 Da
SUBUNIT: Interacts with SGCD (By similarity). Interacts with AGR2 and AGR3.
SUBCELLULAR LOCATION: Alpha-dystroglycan: Secreted, extracellular space. & Beta-dystroglycan: Cell membrane; Single- pass type I membrane protein.
TISSUE SPECIFICITY: Expressed in a variety of fetal and adult tissues.

Molecular Weight

156 kDa

Product Usage Statements
Quality Assurance	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.
Usage Statement	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.

Product Usage Statements

Quality Assurance

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.

Usage Statement

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

Storage and Shipping Information

Storage Conditions

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

Packaging Information
Material Size	200 µL

Packaging Information

Material Size

200 µL

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Anti-a-Dystroglycan, clone IIH6C4

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