Zeitschrift für Glykobiologie
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Pseudomyxoma Peritonei: Uninvited Goblet Cells, Ectopic MUC2

Afshin Amini, Samar Masoumi Moghaddam, Anahid Ehteda and David Lawson Morris*

Pseudomyxoma peritonei (PMP) is a challenging clinical syndrome characterized by multifocal peritoneal collections
of extracellular mucins. Mucins are high molecular weight, heavily glycosylated proteins differentially expressed
by various types of epithelial cells. In this pathological condition, goblet cells originating from a mucinous tumor of the
appendix gain access to the peritoneal cavity where they secrete mucin ectopically. Secreted mucin thus accumulates
and forms the characteristic feature of the disease. Therefore, goblet cells and secreted mucins constitute the two key
elements of the disease. MUC2 is the PMP's specific, predominant mucin. It is a highly viscous, gel-forming mucin
that accounts for the characteristic appearance of PMP mucinous deposits as compared to the mucinous implants of
ovarian origin. Mucin deposits are the real cause of PMP’s morbid complications irrespective of the site of origin, the
mechanism of peritoneal spread, or the level of neoplastic transformation. In this article, role of mucin in gastrointestinal
physiology and PMP pathology are reviewed and the potential of MUC2 as a therapeutic target are discussed.