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Background Rett syndrome ( RTT) is an intellectual deficit and movement disorder that develops during early childhood in girls. Affected children are normal until 6-18 months of age, after which symptoms begin to appear. Most cases of RTT are due to mutations in the Me CP2 gene leading to disruption of neuronal communication in the central nervous system. In addition, RTT patients show peripheral ailments such as gastrointestinal ( GI), respiratory, and cardiac dysfunction. The etiology of intestinal dysfunction in RTT is not well-understood. Reports on the presence of Me CP2 in the peripheral nervous system are scant. As such we examined the levels of Me CP2 in human and murine GI tissue and assessed Me CP2 expression at various developmental stages. Methods Immunohistochemistry for Me CP2, HuC/D, juvenile beta tubulin, and GFAP was performed on human and murine intestine. Western blots of these same tissues were probed with Me CP2, vAChT, nNOS, and beta-actin antibodies. Key Results Me CP2 is expressed throughout the GI tract. Me CP2 is expressed specifically in the enteric nervous system of the GI tract. Me CP2 is expressed in the GI tract throughout development with appearance beginning at or before E11.5 in the murine intestine. Conclusions & Inferences The proof of Me CP2 expression in enteric neurons suggests that the GI dysmotility in Rett may arise from enteric network dysfunction secondary to Me CP2 mutation. [ABSTRACT FROM AUTHOR]