TY - JOUR
T1 - Human autoantibodies against HD1/plectin in paraneoplastic pemphigus
AU - Proby, Charlotte
AU - Fujii, Yoshiko
AU - Owaribe, Katsushi
AU - Nishikawa, Takeji
AU - Amagai, Masayuki
N1 - Funding Information:
We gratefully acknowledge PNP sera provided by Drs. Grant J. Anhalt and Takashi Hashimoto and the anti-desmoplakin monoclonal antibody provided by Dr. David Garrod. We would like to thank Mrs. Minae Suzuki for help with immunoblotting. This work was supported by Grant-In-Aid for Scientific Research from the Ministry of Education, Science, and Culture of Japan, and Research Grants for Life Sciences and Medicine, Keio University Medical Science Fund, and Keio Gijuku Academic Development Funds.
PY - 1999
Y1 - 1999
N2 - Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. PNP patients develop characteristic autoantibodies directed against multiple antigens, mostly identified as members of the plakin family of cytoplasmic proteins (desmoplakin I and II, bullous pemphigoid antigen I, envoplakin, and periplakin). HD1/plectin, another member of the plakin family, has not previously been detected in the characteristic PNP antigen complex, which may relate to practical difficulties associated with its large size (molecular weight ≃ 500 kDa). In this study, a combination of immunoprecipitation and immunoblot is used to demonstrate that HD1/plectin is also recognized by sera from PNP patients. Thirteen of 16 PNP sera tested were positive for HD1/plectin compared with none of 43 control sera (11 pemphigus vulgaris, 11 pemphigus foliaceus, 11 bullous pemphigoid, and 10 normal individuals). Combined with our recent finding that desmoglein 3 and desmoglein 1 are cell surface target antigens in PNP, this demonstration of plectin/HD1 as another component of the antigen complex in PNP confirms that PNP is an autoimmune disease against desmoglein and plakin family molecules.
AB - Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. PNP patients develop characteristic autoantibodies directed against multiple antigens, mostly identified as members of the plakin family of cytoplasmic proteins (desmoplakin I and II, bullous pemphigoid antigen I, envoplakin, and periplakin). HD1/plectin, another member of the plakin family, has not previously been detected in the characteristic PNP antigen complex, which may relate to practical difficulties associated with its large size (molecular weight ≃ 500 kDa). In this study, a combination of immunoprecipitation and immunoblot is used to demonstrate that HD1/plectin is also recognized by sera from PNP patients. Thirteen of 16 PNP sera tested were positive for HD1/plectin compared with none of 43 control sera (11 pemphigus vulgaris, 11 pemphigus foliaceus, 11 bullous pemphigoid, and 10 normal individuals). Combined with our recent finding that desmoglein 3 and desmoglein 1 are cell surface target antigens in PNP, this demonstration of plectin/HD1 as another component of the antigen complex in PNP confirms that PNP is an autoimmune disease against desmoglein and plakin family molecules.
KW - Autoimmunity
KW - Desmoglein
KW - Plakins
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U2 - 10.1046/j.1523-1747.1999.00498.x
DO - 10.1046/j.1523-1747.1999.00498.x
M3 - Article
C2 - 9989789
AN - SCOPUS:0032916947
SN - 0022-202X
VL - 112
SP - 153
EP - 156
JO - Journal of Investigative Dermatology
JF - Journal of Investigative Dermatology
IS - 2
ER -