Dystrophin Polyclonal Antibody | anti-DMD antibody

Anti-Dystrophin Antibody

Cat. Num. AAA177765

• Gene Names: DMD; BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Immunogen Affinity Purified
• Synonyms: Dystrophin; Polyclonal Antibody; Anti-Dystrophin Antibody; Apo dystrophin; BMD; CMD3B; DMD; DMD_HUMAN; Duchenne muscular dystrophy protein; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; Muscular dystrophy Duchenne and Becker types; dystrophin; anti-DMD antibody

• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Purity/Purification: Immunogen Affinity Purified
• Form/Format: Lyophilized. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Sequence Length: 3677

• Applicable Applications for anti-DMD antibody: Western Blot (WB), Immunohistochemistry (IHC) Paraffin
• Application Notes: Western Blot Concentration: 0.1-0.5ug/ml; Immunohistochemistry (IHC) Paraffin Concentration: 0.5-1ug/ml
• Immunogen: E Coli-derived human Dystrophin recombinant protein (Position: H3076-D3404). Human Dystrophin shares 100% amino acid (aa) sequence identity with mouse Dystrophin.
• Ig Type: Rabbit IgG
• Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
• Preparation and Storage: At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquoted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Western Blot (WB)

(Anti- Dystrophin Picoband antibody, MBS177765, Western blottingAll lanes: Anti Dystrophin (MBS177765) at 0.5ug/mlLane 1: SMMC Whole Cell Lysate at 40ugLane 2: HEPA Whole Cell Lysate at 40ugPredicted bind size: 427KDObserved bind size: 427KD )

Immunohistochemistry (IHC)

(Anti- Dystrophin Picoband antibody, MBS177765, IHC(P)IHC(P): Mouse Brain Tissue )

Immunohistochemistry (IHC)

(Anti- Dystrophin Picoband antibody, MBS177765, IHC(P)IHC(P): Rat Cardiac Muscle Tissue )

Immunohistochemistry (IHC)

(Anti- Dystrophin Picoband antibody, MBS177765, IHC(P)IHC(P): Human Lung Cancer Tissue )

Related Product Information for anti-DMD antibody

Description: Rabbit IgG polyclonal antibody for Dystrophin(DMD) detection. Tested with WB, IHC-P in Human;Mouse;Rat.

Background: Dystrophin, also known as DMD, is a rod-shaped cytoplasmic protein, and a vital part of a protein complex that connects the cytoskeleton of a muscle fiber to the surrounding extracellular matrix through the cell membrane. It is mapped to Xp21.2-p21.1. This complex is variously known as the costamere or thedystrophin-associated protein complex. Many muscle proteins, such as alpha-dystrobrevin, syncoilin, synemin, sarcoglycan, dystroglycan, and sarcospan, colocalize with dystrophin at the costamere. Dystrophin is a protein located between the sarcolemma and the outermost layer of myofilaments in the muscle fiber (myofiber). It is a cohesive protein, linking actin filaments to another support protein that resides on the inside surface of each muscle fiber's plasma membrane (sarcolemma).

References

1. Tennyson CN, Klamut HJ, Worton RG (1995). "The human dystrophin gene requires 16 hours to be transcribed and is cotranscriptionally spliced". Nature Genetics 9 (2): 184-90. 2. García-Pelagio KP, Bloch RJ, Ortega A, González-Serratos H (2011). "Biomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin- null mice". J Muscle Res Cell Motil 31 (5-6): 323-336. 3. Strachan T and Read AP, 1999. Human molecular genetics, BIOS Scientific, New York, USA

NCBI and Uniprot Product Information

• NCBI GI #: 5032281
• NCBI GeneID: 1756
• NCBI Accession #: NP_000100.2
• NCBI GenBank Nucleotide #: NM_000109.3
• UniProt Accession #: P11532; Q02295; Q14169; Q14170; Q5JYU0; Q6NSJ9; Q7KZ48; Q8N754; Q9UCW3; Q9UCW4; E9PDN1
• Molecular Weight: 57,953 Da
• NCBI Official Full Name: dystrophin Dp427c isoform
• NCBI Official Synonym Full Names: dystrophin
• NCBI Official Symbol: DMD
• NCBI Official Synonym Symbols: BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
• NCBI Protein Information: dystrophin
• UniProt Protein Name: Dystrophin
• Protein Family: Dystrophin
• UniProt Gene Name: DMD
• UniProt Entry Name: DMD_HUMAN

NCBI Description

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. [provided by RefSeq, Jul 2008]

Uniprot Description

dystrophin: Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin- associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission. Defects in DMD are the cause of Duchenne muscular dystrophy (DMD). 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). BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. Defects in DMD are a cause of cardiomyopathy dilated X- linked type 3B (CMD3B); also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: Xp21.2

Cellular Component: actin cytoskeleton; cell surface; cell-matrix junction; costamere; cytoskeleton; cytosol; dystrophin-associated glycoprotein complex; filopodium; filopodium membrane; lateral plasma membrane; lipid raft; nucleus; plasma membrane; postsynaptic membrane; protein complex; sarcolemma; synapse; syntrophin complex; Z disc

Molecular Function: actin binding; myosin binding; nitric-oxide synthase binding; protein binding; structural constituent of cytoskeleton; structural constituent of muscle; vinculin binding; zinc ion binding

Biological Process: cardiac muscle contraction; cellular protein complex assembly; muscle attachment; muscle development; muscle fiber development; muscle filament sliding; muscle maintenance; negative regulation of peptidyl-serine phosphorylation; peptide biosynthetic process; positive regulation of neuron differentiation; regulation of heart rate; regulation of skeletal muscle contraction; regulation of skeletal muscle contraction via regulation of the release of sequestered calcium ion

Disease: Cardiomyopathy, Dilated, 3b; Muscular Dystrophy, Becker Type; Muscular Dystrophy, Duchenne Type