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Chemical Compound Review

Asulame     methylN-(4- aminophenyl)sulfonylcarbamate

Synonyms: ASULAM, Asilan, Asulox, Jonnix, Plakin, ...
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Disease relevance of ASULAM

  • One member of this group is bullous pemphigoid antigen 1 (Bpag1)/dystonin, which has neuronal and muscle isoforms that consist of actin-binding and microtubule-binding domains at either end separated by a plakin domain and several spectrin repeats [1].
  • The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin [2].
  • The plakin family of linker proteins have been in the spotlight recently as critical components for embryo survival and, when mutated, the cause of diseases such as muscular dystrophy and cardiomyopathies [3].
  • BACKGROUND: In select cases, lichen planus has been observed to be a paraneoplastic condition sometimes associated with paraneoplastic pemphigus, a disease featuring autoantibodies directed against plakin proteins, desmogleins 3 and 1, and a still uncharacterized 170-kd antigen [4].
  • CONCLUSIONS: The presence and localization in tumours of B-lymphocyte clones against proteins of the plakin family and desmoglein 3 in skin may not be confined to PNP with Castleman disease, but is possibly a common mechanism in PNP associated with various tumours [5].

High impact information on ASULAM

  • We have now isolated a third member of the nesprin family that lacks an ABD and instead binds to the plakin family member plectin, which can associate with the intermediate filament (IF) system [6].
  • We find that the plakin repeats are inserted between the actin-binding domain and spectrin repeats, generating isoforms as large as 8,846 residues, which could span 400 nm [7].
  • Our analysis of vab-10 reveals novel insights into the role of this plakin subfamily. vab-10 generates isoforms related either to plectin (termed VAB-10A) or to microtubule actin cross-linking factor plakins (termed VAB-10B) [8].
  • Bullous pemphigoid antigen 1 (BPAG1) is a member of the plakin family with cytoskeletal linker properties [9].
  • BPAG1-b is highly expressed in muscles, and has extra exons encoding a second IFBD between the plakin and SR-containing rod domains of BPAG1-a [9].

Biological context of ASULAM


Anatomical context of ASULAM


Gene context of ASULAM

  • The plakin Short Stop and the RhoA GTPase are required for E-cadherin-dependent apical surface remodeling during tracheal tube fusion [18].
  • Several proteins belonging to the plakin family of cytoskeletal linker proteins have recently been identified, including dystonin/Bpag1 and plectin [19].
  • Dynamics and unexpected localization of the plakin binding protein, kazrin, in mouse eggs and early embryos [3].
  • We have previously reported the cloning and partial characterization of Acf7, a novel member of the plakin family [19].
  • The short C-terminal domain of periplakin is composed only of the linker domain, a region highly homologous between the plakin proteins [20].

Analytical, diagnostic and therapeutic context of ASULAM

  • Immunoblotting of equal amounts of each plakin tail-glutathione S-transferase fusion protein with PNP sera revealed a strong reaction with the envoplakin tail domain [2].
  • Interestingly, indirect immunofluorescence on rat bladder was negative and immunoblot analysis of the patient's serum on epidermal extracts demonstrated antiepidermal antibodies that only recognized the pemphigus vulgaris antigen desmoglein 3, with no antibodies directed against the different proteins of the plakin family [21].


  1. Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. Young, K.G., Pool, M., Kothary, R. J. Cell. Sci. (2003) [Pubmed]
  2. The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. Mahoney, M.G., Aho, S., Uitto, J., Stanley, J.R. J. Invest. Dermatol. (1998) [Pubmed]
  3. Dynamics and unexpected localization of the plakin binding protein, kazrin, in mouse eggs and early embryos. Gallicano, G.I., Foshay, K., Pengetnze, Y., Zhou, X. Dev. Dyn. (2005) [Pubmed]
  4. Lichenoid dermatitis in paraneoplastic pemphigus: a pathogenic trigger of epitope spreading? Bowen, G.M., Peters, N.T., Fivenson, D.P., Su, L.D., Nousari, H.C., Anhalt, G.J., Cooper, K.D., Stevens, S.R. Archives of dermatology. (2000) [Pubmed]
  5. Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. Wang, J., Bu, D.F., Li, T., Zheng, R., Zhang, B.X., Chen, X.X., Zhu, X.J. Br. J. Dermatol. (2005) [Pubmed]
  6. Nesprin-3, a novel outer nuclear membrane protein, associates with the cytoskeletal linker protein plectin. Wilhelmsen, K., Litjens, S.H., Kuikman, I., Tshimbalanga, N., Janssen, H., van den Bout, I., Raymond, K., Sonnenberg, A. J. Cell Biol. (2005) [Pubmed]
  7. Maintaining epithelial integrity: a function for gigantic spectraplakin isoforms in adherens junctions. Röper, K., Brown, N.H. J. Cell Biol. (2003) [Pubmed]
  8. The Caenorhabditis elegans vab-10 spectraplakin isoforms protect the epidermis against internal and external forces. Bosher, J.M., Hahn, B.S., Legouis, R., Sookhareea, S., Weimer, R.M., Gansmuller, A., Chisholm, A.D., Rose, A.M., Bessereau, J.L., Labouesse, M. J. Cell Biol. (2003) [Pubmed]
  9. The BPAG1 locus: Alternative splicing produces multiple isoforms with distinct cytoskeletal linker domains, including predominant isoforms in neurons and muscles. Leung, C.L., Zheng, M., Prater, S.M., Liem, R.K. J. Cell Biol. (2001) [Pubmed]
  10. Kakapo, a novel cytoskeletal-associated protein is essential for the restricted localization of the neuregulin-like factor, vein, at the muscle-tendon junction site. Strumpf, D., Volk, T. J. Cell Biol. (1998) [Pubmed]
  11. Structure and regulation of the envoplakin gene. Määttä, A., Ruhrberg, C., Watt, F.M. J. Biol. Chem. (2000) [Pubmed]
  12. Role of binding of plectin to the integrin beta4 subunit in the assembly of hemidesmosomes. Koster, J., van Wilpe, S., Kuikman, I., Litjens, S.H., Sonnenberg, A. Mol. Biol. Cell (2004) [Pubmed]
  13. Structural Analysis of the Plakin Domain of Bullous Pemphigoid Antigen1 (BPAG1) Suggests that Plakins Are Members of the Spectrin Superfamily. Jefferson, J.J., Ciatto, C., Shapiro, L., Liem, R.K. J. Mol. Biol. (2007) [Pubmed]
  14. An epidermal plakin that integrates actin and microtubule networks at cellular junctions. Karakesisoglou, I., Yang, Y., Fuchs, E. J. Cell Biol. (2000) [Pubmed]
  15. Periplakin, a novel component of cornified envelopes and desmosomes that belongs to the plakin family and forms complexes with envoplakin. Ruhrberg, C., Hajibagheri, M.A., Parry, D.A., Watt, F.M. J. Cell Biol. (1997) [Pubmed]
  16. Periplakin gene targeting reveals a constituent of the cornified cell envelope dispensable for normal mouse development. Aho, S., Li, K., Ryoo, Y., McGee, C., Ishida-Yamamoto, A., Uitto, J., Klement, J.F. Mol. Cell. Biol. (2004) [Pubmed]
  17. Gene targeting of envoplakin, a cytoskeletal linker protein and precursor of the epidermal cornified envelope. Määttä, A., DiColandrea, T., Groot, K., Watt, F.M. Mol. Cell. Biol. (2001) [Pubmed]
  18. The plakin Short Stop and the RhoA GTPase are required for E-cadherin-dependent apical surface remodeling during tracheal tube fusion. Lee, S., Kolodziej, P.A. Development (2002) [Pubmed]
  19. Acf7 (MACF) is an actin and microtubule linker protein whose expression predominates in neural, muscle, and lung development. Bernier, G., Pool, M., Kilcup, M., Alfoldi, J., De Repentigny, Y., Kothary, R. Dev. Dyn. (2000) [Pubmed]
  20. Unique role for the periplakin tail in intermediate filament association: specific binding to keratin 8 and vimentin. Kazerounian, S., Uitto, J., Aho, S. Exp. Dermatol. (2002) [Pubmed]
  21. Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3. Bouloc, A., Joly, P., Saint-Leger, E., Wechsler, J., Roujeau, J.C., Revuz, J. J. Am. Acad. Dermatol. (2000) [Pubmed]
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