Desmoplakin (DSP) (C-term) Mouse Monoclonal Antibody [Clone ID: DPI/II 236.23.1]
CAT#: AM09122SU-N
Desmoplakin (DSP) (C-term) mouse monoclonal antibody, clone DPI/II 236.23.1, Supernatant
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CNY 4,895.00
货期*
5周
规格
Cited in 2 publications. |
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Specifications
Product Data | |
Clone Name | DPI/II 236.23.1 |
Applications | IF, IHC, WB |
Recommend Dilution | Immunoblotting. Immunofluorescence. Immunohistochemistry on Frozen Sections. Immunohistochemistry on Paraffin Embedded Sections (Microwave retrieval method required). Working Dilution: Ready to Use for Immunohistochemistry. Incubation Time: 1 hour at RT. |
Reactivity | Bovine, Human, Mouse, Xenopus |
Host | Mouse |
Clonality | Monoclonal |
Immunogen | C-terminal polypeptide of recombinant Human Desmoplakin (starting at amino acid position 1980) |
Specificity | The antibody shows distinct punctate membrane staining of different epithelia. Desmosomes of the myocardium are also positive. Tumors Specifically Detected: Primary and metastatic carcinoma and meningioma. Tested Reactivities on Cultured Cell Lines: Several human carcinoma cell lines: MCF-7, A-431, HaCaT, CaCo2; bovine cells: MDBK, BMGE. Polypeptides Reacting: Desmoplakin 1 and 2 (Mr 250 000 and 215 000). |
Formulation | State: Supernatant State: Liquid Culture Supernatant Preservative: 0.09% Sodium Azide |
Conjugation | Unconjugated |
Storage Condition | Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. |
Gene Name | desmoplakin |
Database Link | |
Background | Desmosomes are intercellular junctions that form tight links between adjacent cells. Desmoplakin is an obligate component of functional desmosomes that attaches intermediate filaments to desmosomal plaques. It is involved in the organization of desmosomal cadherin-plakoglobin complexes into discrete plasma membrane domains. The N-terminus of desmoplakin is essential for localisation to the desmosome and interaction with plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds to intermediate filaments. The central region of desmoplakin comprises a coiled-coil rod domain that mediates homodimerisation. There are two isoforms of desmoplakin - desmoplakin I, which is an obligate component of all desmosomes, and desmoplakin II, which is predominantly expressed in tissues and cells of stratified origin. Mutations in the gene encoding desmoplakin result in a number of cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus. |
Synonyms | DP, DSP, 250/210 kDa paraneoplastic pemphigus antigen |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Arrhythmogenic right ventricular cardiomyopathy (ARVC) |
Citations (2)
The use of this Antibodies has been cited in the following citations: |
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A Scandinavian case of skin fragility, alopecia and cardiomyopathy caused by DSP mutations.
,null,
Clinical and experimental dermatology
,PubMed ID 24341478
[DSP]
|
The novel desmin mutant p.A120D impairs filament formation, prevents intercalated disk localization, and causes sudden cardiac death
,Brodehl A, Dieding M, Klauke B, Dec E, Madaan S, Huang T, Gargus J, Fatima A, Saric T, Cakar H, Walhorn V, Tvnsing K, Skrzipczyk T, Cebulla R, Gerdes D, Schulz U, Gummert J, Svendsen JH, Olesen MS, Anselmetti D, Christensen AH, Kimonis V, Milting H,
Circ Cardiovasc Genet
,PubMed ID 24200904
[DSP]
|
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