Mouse monoclonal antibody to human Dystrophin, [MANDYS8] Ascites

ZZZZ_Status (Active, Disc) : Active

 

AA_Supplier Name : Biosensis

 

Alternative_Name (Other Names) :

DMD

 

Antibody_Isotype
(Antibody only) : IgG2b

 

Application_Details :

Immunohistochemistry (IHC) and Western Blotting (WB). A 1:200-1:1:000 is recommended for WB. Human dystrophin has a predicted length of 3,685 residues and
MW of 427 kDa. A concentration of 1:200-1:6000 (ABC detection) is
recommended to detect dystrophin fresh frozen, or acetone fixed, 2%
PLP fixed sections so far tested. Not recommended for paraffin
embedded tissues. Immunolocalization: Stains muscle membranes (no
staining of Duchenne muscle membrane). Biosensis recommends
optimal dilutions/concentrations should be determined by the end user.

 

Background_Info : 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..
In muscle cells, sarcolemma localization requires the presence of ANK2, while localization to costameres requires the presence of ANK3. Localizes to neuromuscular junctions (NMJs). In adult muscle, NMJ localization depends upon ANK2 presence, but not in newborn animals.
Involvement in diseasei
Duchenne muscular dystrophy (DMD)6 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionMost 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.
Source: https://www.uniprot.org/uniprot/P11532

 

Clone_Name
(AB only, monoclonals) : 8H11/MANDYS8

 

Datasheet_Link : http://www.biosensis.com/pdf_datasheet.php?products_id=1670

 

Description (Long Description) : 

Dystrophin is a large cytoskeletal protein (426 kDa) found at the inner surface of muscle fibres. Dystrophin is part of the dystrophin glycoprotein complex (DGC), which bridges the inner cytoskeleton (F actin) and the extracellular matrix. The monoclonal antibody clone MANDYS8 was developed in K.E.Davies’ laboratory (see Nguyen Thi Man et al. 1990). This mouse monoclonal specifically recognizes an epitope located on the rod domain of the human dystrophin molecule. The antibody reacts with dystrophin from human, mouse, rat, rabbit, and porcine, but not canine or frog origin. This epitope is present in normal muscle tissue and in nearly all Becker muscular dystrophies. It is absent in cases of Duchenne muscular dystrophies and in the dystrophic mouse (mdx). The epitope recognized by the antibody is sensitive to formalin fixation and paraffin embedding.

 

Expiry_Date : 

12 months after purchase.

 

Format (Form) : 

Lyophilized from 1.2% sodium acetate, 2mg BSA, 0.01mg NaN3

 

Immunogen (Antigen) :Recombinant human dystrophin encoding amino-acids 816-1749

 

Kit_Components :

 

MSDS (File name) :