Anti-a-Dystroglycan, clone VIA4-1

Species Reactivity	Key Applications	Host	Format	Antibody Type
Ca, Gp, H, M, R, Rb	IHC, WB	M	Culture Supernatant	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

Ca, Gp, H, M, R, Rb

IHC, WB

Culture Supernatant

Monoclonal Antibody

Description
Catalogue Number	05-298
Brand Family	Upstate
Trade Name	Upstate
Description	Anti-α-Dystroglycan Antibody, clone VIA4-1
Overview	Note: 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.
Background Information	Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens by chromosome 3. There are two exons that are separated by a large intron. The spliced exons codes for a protein product is finally cleaved into two non-covalently associated subunits, α (N-terminal) and β (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. α-dystroglycan is extracellular and binds to merosin (α-2 laminin) in the basement membrane, while β-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence that suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either α- or β-dystroglycan. Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear.

Description

Catalogue Number

05-298

Brand Family

Upstate

Trade Name

Upstate

Description

Anti-α-Dystroglycan Antibody, clone VIA4-1

Overview

Note: 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.

Background Information

Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens by chromosome 3. There are two exons that are separated by a large intron. The spliced exons codes for a protein product is finally cleaved into two non-covalently associated subunits, α (N-terminal) and β (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. α-dystroglycan is extracellular and binds to merosin (α-2 laminin) in the basement membrane, while β-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence that suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either α- or β-dystroglycan.
Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear.

Product Information
Format	Culture Supernatant
Control	Mouse, rat and human skeletal muscle tissue extracts or DU 145 (human prostate carcinoma tumor) cell lysate
Presentation	Culture supernatant containing 0.05% sodium azide.

Product Information

Format

Culture Supernatant

Control

Mouse, rat and human skeletal muscle tissue extracts or DU 145 (human prostate carcinoma tumor) cell lysate

Presentation

Culture supernatant containing 0.05% sodium azide.

Applications
Application	Detect α-Dystroglycan using this Anti-α-Dystroglycan Antibody, clone VIA4-1. This antibody has been published and validated for use in IH and WB.
Key Applications	Immunohistochemistry Western Blotting

Applications

Application

Detect α-Dystroglycan using this Anti-α-Dystroglycan Antibody, clone VIA4-1. This antibody has been published and validated for use in IH and WB.

Key Applications

Immunohistochemistry
Western Blotting

Biological Information
Immunogen	Rabbit skeletal muscle membrane preparation
Clone	VIA4-1
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Mouse
Specificity	Recognizes α-dystroglycan/LARGE-glycan.
Isotype	IgG1
Species Reactivity	Canine Guinea Pig Human Mouse Rat Rabbit
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

VIA4-1

Concentration

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

Host

Mouse

Specificity

Recognizes α-dystroglycan/LARGE-glycan.

Isotype

IgG1

Species Reactivity

Canine
Guinea Pig
Human
Mouse
Rat
Rabbit

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 on rabbit skeletal muscle in a western blot.
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 on rabbit skeletal muscle in a western blot.

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	Maintain for 2 years at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Storage and Shipping Information

Storage Conditions

Maintain for 2 years at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and 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 VIA4-1

浏览了该商品的用户最终购买

浏览了该商品的用户还浏览了

特卖

重要规格表

我要咨询