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).
Alternative splicing generates two isoforms (DP I and DP II) that differ in their rod domain lengths, with relative molecular weights of 332 kD and 259 kD, respectively (Green et al.
1988).
Mutations in desmoplakin can cause cardiocutaneous syndromes (for review, see Cheong et al.
2005).
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).
Applications:Suitable for use in Immunohistochemistry (frozen tissue and for paraffin-embedded tissue, improved when using the microwave method) and Immunoblot (Western).
Other applications not tested.
Recommended Dilution:Immunohistochemistry: 1:100 (frozen); Incubation Time 1 hr at RT; extended with paraffin sectionsImmunohistochemistry: 1:50 (paraffin-embedded tissue)Western Blot: 1:1000Optimal dilution determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：guinea pig
Source Antibody：bovine
Grade：Serum
Purity：Serum
Form：Supplied as a liquid.
Specificity：Shows distinct punctate membrane staining of epithelia. In Western blot analysis the antiserum reacts specifically with the desmoplakin I polypeptide.
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Bo
Immunogen：Bovine desmoplakin 1 (prepared from SDS-polyacryl- amide gels).
