Anti-Dystrophin (Mouse Monoclonal)

Species Reactivity	Key Applications	Host	Format	Antibody Type
Ch, H, M, R	WB, IHC	M	Purified	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

Ch, H, M, R

WB, IHC

Purified

Monoclonal Antibody

Description
Catalogue Number	MAB1645
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-Dystrophin Antibody, clone 1808

Description

Catalogue Number

MAB1645

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-Dystrophin Antibody, clone 1808

Product Information
Format	Purified
Control	POSITIVE CONTROL: Snap frozen normal human or rat striated muscle.
Presentation	Liquid in 0.02 M phosphate buffer (pH 7.6), 250 mM NaCl with 0.1% sodium azide.

Product Information

Format

Purified

Control

POSITIVE CONTROL: Snap frozen normal human or rat striated muscle.

Presentation

Liquid in 0.02 M phosphate buffer (pH 7.6), 250 mM NaCl with 0.1% sodium azide.

Applications
Application	Anti-Dystrophin Antibody, clone 1808 detects level of Dystrophin & has been published & validated for use in WB, IH.
Key Applications	Western Blotting Immunohistochemistry
Application Notes	Western blot Immunohistochemistry Optimal working dilutions must be determined by the end user.

Applications

Application

Anti-Dystrophin Antibody, clone 1808 detects level of Dystrophin & has been published & validated for use in WB, IH.

Key Applications

Western Blotting
Immunohistochemistry

Application Notes

Western blot

Immunohistochemistry

Optimal working dilutions must be determined by the end user.

Biological Information
Immunogen	Peripheral membrane proteins extracted from Torpedo postsynaptic membranes.
Clone	1808
Host	Mouse
Specificity	By Western blot MAB1645 recognizes a single protein having a relative molecular weight of 300 kD. Reacts to mammalian skeletal muscle dystrophin. Shows no staining of mdx mouse muscle. Labels normal human muscle but not Duchenne muscle. STAINING PATTERN: On cryostat sections of normal mouse diaphragm MAB1645 gives strong immunofluorescence staining of the entire sarcolemma, with particularly strong staining of neuromuscular junctions. SPECIES REACTIVITY: Labels skeletal muscle from rat, chicken and Xenopus laevis. Labels cardiac muscle from rat and normal, but not mdx, mouse. Also labels smooth muscle from chicken gizzard.
Isotype	IgG1
Species Reactivity	Chicken Human Mouse Rat
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_000109.2 NM_004006.1 NM_004007.1 NM_004009.2 NM_004010.2 NM_004011.2 NM_004012.2 NM_004013.1 NM_004014.1 NM_004015.1 NM_004016.1 NM_004017.1 NM_004018.1 NM_004019.2 NM_004020.1 NM_004021.1 NM_004022.1 NM_004023.1
Entrez Gene Summary	The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix.
Gene Symbol	DMD DXS272 DXS269 DXS230 Dystrophin. DXS268 DXS270 DXS239 DXS142 CMD3B DXS206 dystrophin DXS164 BMD
UniProt Number	P11532
UniProt Summary	FUNCTION: SwissProt: P11532 # May play a role in anchoring the cytoskeleton to the plasma membrane. SIZE: 3685 amino acids; 426692 Da SUBUNIT: Interacts with the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Interacts with KRT19. SUBCELLULAR LOCATION: Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. TISSUE SPECIFICITY: Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Isoform 5 is expressed in brain, liver, testis and hepatoma cells. DISEASE: SwissProt: P11532 # Defects in DMD are the cause of Duchenne muscular dystrophy (DMD) [MIM:310200]. DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. & Defects in DMD are the cause of Becker muscular dystrophy (BMD) [MIM:300376]. BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. & Defects in DMD are a cause of dilated cardiomyopathy [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dystrophin mutations may predispose to common sporadic cardiomyopathy cases. SIMILARITY: SwissProt: P11532 ## Contains 2 CH (calponin-homology) domains. & Contains 22 spectrin repeats. & Contains 1 WW domain. & Contains 1 ZZ-type zinc finger. MISCELLANEOUS: The DMD gene is the largest known gene in humans. It is 2.4 million base-pairs in size, comprises 79 exons and takes over 16 hours to be transcribed and cotranscriptionally spliced.

Biological Information

Immunogen

Peripheral membrane proteins extracted from Torpedo postsynaptic membranes.

Clone

1808

Host

Mouse

Specificity

By Western blot MAB1645 recognizes a single protein having a relative molecular weight of 300 kD. Reacts to mammalian skeletal muscle dystrophin. Shows no staining of mdx mouse muscle. Labels normal human muscle but not Duchenne muscle.

STAINING PATTERN:

On cryostat sections of normal mouse diaphragm MAB1645 gives strong immunofluorescence staining of the entire sarcolemma, with particularly strong staining of neuromuscular junctions.

SPECIES REACTIVITY:

Labels skeletal muscle from rat, chicken and Xenopus laevis. Labels cardiac muscle from rat and normal, but not mdx, mouse. Also labels smooth muscle from chicken gizzard.

Isotype

IgG1

Species Reactivity

Chicken
Human
Mouse
Rat

Antibody Type

Monoclonal Antibody

Entrez Gene Number

Entrez Gene Summary

The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix.

Gene Symbol

DMD
DXS272
DXS269
DXS230
Dystrophin.
DXS268
DXS270
DXS239
DXS142
CMD3B
DXS206
dystrophin
DXS164
BMD

UniProt Number

P11532

UniProt Summary

FUNCTION: SwissProt: P11532 # May play a role in anchoring the cytoskeleton to the plasma membrane.
SIZE: 3685 amino acids; 426692 Da
SUBUNIT: Interacts with the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Interacts with KRT19.
SUBCELLULAR LOCATION: Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton.
TISSUE SPECIFICITY: Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Isoform 5 is expressed in brain, liver, testis and hepatoma cells.
DISEASE: SwissProt: P11532 # Defects in DMD are the cause of Duchenne muscular dystrophy (DMD) [MIM:310200]. DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. & Defects in DMD are the cause of Becker muscular dystrophy (BMD) [MIM:300376]. BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. & Defects in DMD are a cause of dilated cardiomyopathy [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dystrophin mutations may predispose to common sporadic cardiomyopathy cases.
SIMILARITY: SwissProt: P11532 ## Contains 2 CH (calponin-homology) domains. & Contains 22 spectrin repeats. & Contains 1 WW domain. & Contains 1 ZZ-type zinc finger.
MISCELLANEOUS: The DMD gene is the largest known gene in humans. It is 2.4 million base-pairs in size, comprises 79 exons and takes over 16 hours to be transcribed and cotranscriptionally spliced.

Product Usage Statements
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

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 at -20ºC for up to 12 months in convenient aliquots. Avoid repeated freeze/thaw cycles.

Storage and Shipping Information

Storage Conditions

Maintain at -20ºC for up to 12 months in convenient aliquots. Avoid repeated freeze/thaw cycles.

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-Dystrophin (Mouse Monoclonal)

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