title: Expression of Cystic Fibrosis Transmembrane Conductance Regulator in Ganglia of Human Gastrointestinal Tract.
abstract: CF is caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) which is an anion selective transmembrane ion channel that mainly regulates chloride transport, expressed in the epithelia of various organs. Recently, we have demonstrated CFTR expression in the brain, the spinal cord and the sympathetic ganglia. This study aims to investigate the expression and distribution of CFTR in the ganglia of the human gastrointestinal tract. Fresh tissue and formalin-fixed paraffin-embedded normal gastrointestinal tract samples were collected from eleven surgical patients and five autopsy cases. Immunohistochemistry, in situ hybridization, laser-assisted microdissection and nested reverse transcriptase polymerase chain reaction were performed. Expression of CFTR protein and mRNA was detected in neurons of the ganglia of all segments of the human gastrointestinal tract examined, including the stomach, duodenum, jejunum, ileum, cecum, appendix, colon and rectum. The extensive expression of CFTR in the enteric ganglia suggests that CFTR may play a role in the physiology of the innervation of the gastro-intestinal tract. The presence of dysfunctional CFTRs in enteric ganglia could, to a certain extent, explain the gastrointestinal symptoms frequently experienced by CF patients.
authors.affiliation: Department of Pathology, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, China.
authors.forename: Ruiqi
authors.initials: R
authors.lastname: Xue
authors.affiliation: Department of Pathology, Key Laboratory of Infectious Diseases and Molecular Pathology, Guangdong Province, Collaborative and Creative Center of Molecular Pathology and Personalized Medicine, Shantou University Medical College, Shantou, China.|Department of Physics, University of Maryland, College Park, MD, USA.
authors.forename: Huan
authors.initials: H
authors.lastname: Gu
authors.affiliation: Department of Pathology, Key Laboratory of Infectious Diseases and Molecular Pathology, Guangdong Province, Collaborative and Creative Center of Molecular Pathology and Personalized Medicine, Shantou University Medical College, Shantou, China.
authors.forename: Yamei
authors.initials: Y
authors.lastname: Qiu
authors.affiliation: Department of Pathology, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Guo
authors.affiliation: Department of Pathology, Key Laboratory of Infectious Diseases and Molecular Pathology, Guangdong Province, Collaborative and Creative Center of Molecular Pathology and Personalized Medicine, Shantou University Medical College, Shantou, China.
authors.forename: Christine
authors.initials: C
authors.lastname: Korteweg
authors.affiliation: Department of Pathology, Key Laboratory of Infectious Diseases and Molecular Pathology, Guangdong Province, Collaborative and Creative Center of Molecular Pathology and Personalized Medicine, Shantou University Medical College, Shantou, China.
authors.forename: Jin
authors.initials: J
authors.lastname: Huang
authors.affiliation: Department of Pathology, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, China.|Department of Pathology, Key Laboratory of Infectious Diseases and Molecular Pathology, Guangdong Province, Collaborative and Creative Center of Molecular Pathology and Personalized Medicine, Shantou University Medical College, Shantou, China.
authors.forename: Jiang
authors.initials: J
authors.lastname: Gu
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D003550:Cystic Fibrosis / Q000188:drug therapy*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D005260:Female; D005724:Ganglia / Q000473:pathology*; D005786:Gene Expression Regulation; D006801:Humans; D007150:Immunohistochemistry; D007413:Intestinal Mucosa / Q000378:metabolism*; D007422:Intestines / Q000294:innervation; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000502:physiology*
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator