Desmoplakin 1

Desmoplakin (DP) is a cytoplasmic plaque protein that is located in desmosomes, which are abundant in tissues subjected to mechanical stress, like muscle and epidermis. The role of DP is to link the transmembrane cadherins via plakoglobin to the cytoplasmic intermediate filament network (Kowalczyk et al. 1997; Smith and Fuchs 1998). DP comprises an N-terminal, desmosome-associated plakin domain binding to plakoglobin and plakophilin (Smith and Fuchs 1998), a central coiled-coil rod domain responsible for dimerization, and a C-terminal domain consisting of three homologous plakin repeat subdomains, named A, B, and C, that interact with the intermediate filaments (Choi et al. 2002). Mutations in desmoplakin can cause cardiocutaneous syndromes. Haplotype insufficiency of DP may cause striate palmoplantar keratoderma (PPK [MIM 125647]) (Armstrong et al. 1999; Whittock et al. 1999), whereas dominant missense mutations in the N-terminal domain may cause arrhythmogenic right ventricular dysplasia (ARVD [MIM 607450]) (Rampazzo et al. 2002). A nonsense mutation in the N-terminal domain combined with a recessive missense mutation causes skin fragility/woolly hair syndrome with striate PPK [MIM 607655] (Whittock et al. 2002), whereas a homozygous recessive missense mutation in the B-subdomain causes ARVD, woolly hair, and acral skin blistering (Naxos-like disease) (Alcalai et al. 2003), and a homozygous truncatious mutation in the C-terminus causes left ventricular cardiomyopathy, woolly hair, and PPK (Carvajal syndrome [MIM 605676]) (Norgett et al. 2000). DP must be indispensable, because DP-/- mice are early abortive (Gallicano et al. 1998).\nApplications: \nSuitable for use in Western Blot and Immunohistochemistry. Other applications have not been tested. \nRecommended Dilution:\nImmunohistochemistry (Frozen, paraffin): 1:10 diluted with PBS, pH 7.4; Incubate for 1h at RT. Microwave treatment required. \nOptimal dilution determined by the researcher. \nStorage and Stability:\nLyophilized powder may be stored at -20 degrees C. Stable for 12 months at -20 degrees C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Reconstituted product is stable for 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

D3221-89K

Size

50ug

Applications

IHC, WB

Hosts

Mouse

Reactivities

Hum, Mouse, Rat, Bov, Ch/Bird

Form

Supplied as a lyophilized powder from PBS, pH 7.4, 0.5% BSA, 0.09% sodium azide. Reconstitute with 1ml sterile ddH2O.

P Type

Mab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG1

References

Cowin, P., Kapprell, H.P. and Franke, W.W. The comlement of desmosomal plaque proeins in different cell types. J. Cell Biol. 101, 1442-1454 (1985). Franke, W.W., Cowin, P., Grund, C., Kuhn, C. and Kapprell, H.P.: The endothelial junction. The plaque and its components. In: Endothelial Cell Biology. N. Simionescu and M. Simionescu, eds., Plenum Publishing Corp., New York, pp. 147-165 (1988). Moll, I. and Moll, R., Comparative cytokeratin analysis of sweat gland ducts and eccrine poromas. Arch Dermatol Res, 283, 300-309 (1991). Moll, R., Cowin, P., Kapprell, H.P. and Franke, W.W.: Desmosomal proteins: New markers for identification and classification of tumors. Lab. Invest. 54, 4-25 (1986) Schmelz, M. and Franke W.W.: Complexus adhaerentes, a new group of desmoplakin-containing junctions in endothelial cells: The syndesmos connecting retothelial cells of lymph nodes. Eur. J. Cell Biol. 61, 274-289 (1993) Marcel F. Jonkman,et al., Am J Hum Genet. October 2005; 77: 653

Additional Info

Recognizes bovine Desmoplakin 1 at ~250kD; reacts with the rod fragment of desmoplakin 1. Species Crossreactivity: human, rat, chicken, mouse. Reactivity on cultured human carcinoma cell lines: MCF-7, A-431, TR-146; bovine cells: MDBK, BMGE. Specifically detects tumors on primary and metastatic carcinoma, and meningioma. Shows distinct punctate membrane staining of epithelia.