Anti-Alpha-dystrobrevin (rabbit polyclonal)

Species Reactivity	Key Applications	Host	Format	Antibody Type
H, M	WB	Rb	Affinity Purified	Polyclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

H, M

Affinity Purified

Polyclonal Antibody

Description
Catalogue Number	ABN61
Description	Anti-Alpha-dystrobrevin Antibody
Alternate Names	Dystrobrevin alpha DTN-A Alpha-dystrobrevin Dystrophin-related protein 3
Background Information	Alpha-dystrobrevin belongs to the dystropohin family and is a cytoplasmic component of the dystrophin-associated protein complex. The complex localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Defects in Alpha-dystrobrevin arrests myocardial morphogenesis leading to left ventricular non-compaction type 1. This disorder is characterized by a hypertrophic left ventricule with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, it is associated with other congenital heart anomalies such as ventricular septal defects, pulmonic stenosis and atrial septal defects. The right ventricle may also be affected.

Description

Catalogue Number

ABN61

Description

Anti-Alpha-dystrobrevin Antibody

Alternate Names

Dystrobrevin alpha
DTN-A
Alpha-dystrobrevin
Dystrophin-related protein 3

Background Information

Alpha-dystrobrevin belongs to the dystropohin family and is a cytoplasmic component of the dystrophin-associated protein complex. The complex localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Defects in Alpha-dystrobrevin arrests myocardial morphogenesis leading to left ventricular non-compaction type 1. This disorder is characterized by a hypertrophic left ventricule with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, it is associated with other congenital heart anomalies such as ventricular septal defects, pulmonic stenosis and atrial septal defects. The right ventricle may also be affected.

Product Information
Format	Affinity Purified
Control	Human brain tissue lysate
Presentation	Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Product Information

Format

Affinity Purified

Control

Human brain tissue lysate

Presentation

Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Applications
Application	Anti-Alpha-dystrobrevin Antibody detects level of Alpha-dystrobrevin & has been published & validated for use in WB.
Key Applications	Western Blotting

Applications

Application

Anti-Alpha-dystrobrevin Antibody detects level of Alpha-dystrobrevin & has been published & validated for use in WB.

Key Applications

Western Blotting

Biological Information
Immunogen	GST-tagged recombinant protein corresponding to human Alpha-dystrobrevin.
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Rabbit
Species Reactivity	Human Mouse
Species Reactivity Note	Demonstrated to react with Human and Mouse. No homology to Rat was found.
Antibody Type	Polyclonal Antibody
Entrez Gene Number	NP_001381
Entrez Gene Summary	The protein encoded by this gene belongs to the dystrobrevin subfamily of the dystrophin family. This protein is a component of the dystrophin-associated protein complex (DPC), which consists of dystrophin and several integral and peripheral membrane proteins, including dystroglycans, sarcoglycans, syntrophins and alpha- and beta-dystrobrevin. The DPC localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Mutations in this gene are associated with left ventricular noncompaction with congenital heart defects. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jul 2008].
Gene Symbol	DTNA DRP3
Purification Method	Affinity Purfied
UniProt Number	Q9Y4J8
UniProt Summary	FUNCTION: May be involved in the formation and stability of synapses as well as being involved in the clustering of nicotinic acetylcholine receptors. SUBUNIT STRUCTURE: Interacts with dystrophin, utrophin and the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Isoform 7 and isoform 8 do not interact with dystrophin. Binds dystrobrevin binding protein 1. Interacts with MAGEE1 (By similarity). SUBCELLULAR LOCATION: Cytoplasm. Cell junction › synapse. Cell membrane (By similarity). Note: In peripheral nerves, co-localizes with MAGEE1 in the Schwann cell membrane (By similarity). TISSUE SPECIFICITY: Highly expressed in brain, skeletal and cardiac muscles, and expressed at lower levels in lung, liver and pancreas. Isoform 2 is not expressed in cardiac muscle. Isoform 7 and isoform 8 are only expressed in muscle. DOMAIN: The coiled coil domain mediates the interaction with dystrophin and utrophin (By similarity). PTM: Phosphorylation of DTN-1 on tyrosine kinase substrate domain present in the C-terminus (By similarity). INVOLVEMENT IN DISEASE: Defects in DTNA are the cause of left ventricular non-compaction type 1 (LVNC1) [MIM:604169]. Left ventricular non-compaction is due to an arrest of myocardial morphogenesis. The disorder is characterized by a hypertrophic left ventricule with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, it is associated with other congenital heart anomalies such as ventricular septal defects, pulmonic stenosis and atrial septal defects. The right ventricle may also be affected. SEQUENCE SIMILARITIES: Belongs to the dystrophin family. Dystrobrevin subfamily. Contains 1 ZZ-type zinc finger.
Molecular Weight	~45/80 kDa observed. Uniprot describes 8 isoforms produced by alternative splicing ranging from 22-84 kDa. Uncharacterized bands may appear at ~18 and ~57 kDa in some lysates.

Biological Information

Immunogen

GST-tagged recombinant protein corresponding to human Alpha-dystrobrevin.

Concentration

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

Host

Rabbit

Species Reactivity

Human
Mouse

Species Reactivity Note

Demonstrated to react with Human and Mouse. No homology to Rat was found.

Antibody Type

Polyclonal Antibody

Entrez Gene Number

NP_001381

Entrez Gene Summary

The protein encoded by this gene belongs to the dystrobrevin subfamily of the dystrophin family. This protein is a component of the dystrophin-associated protein complex (DPC), which consists of dystrophin and several integral and peripheral membrane proteins, including dystroglycans, sarcoglycans, syntrophins and alpha- and beta-dystrobrevin. The DPC localizes to the sarcolemma and its disruption is associated with various forms of muscular dystrophy. Mutations in this gene are associated with left ventricular noncompaction with congenital heart defects. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jul 2008].

Gene Symbol

DTNA
DRP3

Purification Method

Affinity Purfied

UniProt Number

Q9Y4J8

UniProt Summary

FUNCTION: May be involved in the formation and stability of synapses as well as being involved in the clustering of nicotinic acetylcholine receptors.

SUBUNIT STRUCTURE: Interacts with dystrophin, utrophin and the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Isoform 7 and isoform 8 do not interact with dystrophin. Binds dystrobrevin binding protein 1. Interacts with MAGEE1 (By similarity).

SUBCELLULAR LOCATION: Cytoplasm. Cell junction › synapse. Cell membrane (By similarity). Note: In peripheral nerves, co-localizes with MAGEE1 in the Schwann cell membrane (By similarity).

TISSUE SPECIFICITY: Highly expressed in brain, skeletal and cardiac muscles, and expressed at lower levels in lung, liver and pancreas. Isoform 2 is not expressed in cardiac muscle. Isoform 7 and isoform 8 are only expressed in muscle.

DOMAIN: The coiled coil domain mediates the interaction with dystrophin and utrophin (By similarity).

PTM: Phosphorylation of DTN-1 on tyrosine kinase substrate domain present in the C-terminus (By similarity).

INVOLVEMENT IN DISEASE: Defects in DTNA are the cause of left ventricular non-compaction type 1 (LVNC1) [MIM:604169]. Left ventricular non-compaction is due to an arrest of myocardial morphogenesis. The disorder is characterized by a hypertrophic left ventricule with deep trabeculations and with poor systolic function, with or without associated left ventricular dilation. In some cases, it is associated with other congenital heart anomalies such as ventricular septal defects, pulmonic stenosis and atrial septal defects. The right ventricle may also be affected.

SEQUENCE SIMILARITIES: Belongs to the dystrophin family. Dystrobrevin subfamily.

Contains 1 ZZ-type zinc finger.

Molecular Weight

~45/80 kDa observed. Uniprot describes 8 isoforms produced by alternative splicing ranging from 22-84 kDa. Uncharacterized bands may appear at ~18 and ~57 kDa in some lysates.

Product Usage Statements
Quality Assurance	Evaluated by Western Blot in human brain tissue lysate. Western Blot Analysis: 1 µg/mL of this antibody detected Alpha-dystrobrevin on 10 µg of human brain tissue lysate.
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

Evaluated by Western Blot in human brain tissue lysate.

Western Blot Analysis: 1 µg/mL of this antibody detected Alpha-dystrobrevin on 10 µg of human brain tissue lysate.

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 2-8°C from date of receipt.

Storage and Shipping Information

Storage Conditions

Stable for 1 year at 2-8°C from date of receipt.

Packaging Information
Material Size	100 µg

Packaging Information

Material Size

100 µg

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-Alpha-dystrobrevin (rabbit polyclonal)

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