title: Cystic diseases of the kidney: role of adhesion molecules in normal and abnormal tubulogenesis.
abstract: This short review summarizes some information concerning what is known about matrix adhesion molecules, focal adhesion proteins, and cell-cell adhesion molecules in normal renal development and cystic diseases of the kidney. The focus is on human nephrogenesis and disease, but utilizes critical information gained from genetically manipulated mouse models. Interestingly, a significant role for the human PKD-1-encoded gene product, polycystin-1, has been found in cell-matrix interactions via integrins during development, and mutations lead to autosomal dominant polycystic kidney disease (ADPKD). Recent studies on human ADPKD have implicated polycystin-1 in the formation of multiprotein complexes containing focal adhesion proteins at the basal cell surface of the normal ureteric bud. Further evidence of a critical role of cell-matrix interactions via focal adhesion complex formation is provided by the development of renal cystic disease in tensin knockout mice.
authors.affiliation: Mount Sinai School of Medicine, New York, N.Y., USA. pat.wilson@SMTPlink.mssm.edu
authors.forename: P D
authors.initials: PD
authors.lastname: Wilson
authors.forename: C R
authors.initials: CR
authors.lastname: Burrow
mesh_terms: D000818:Animals; D002448:Cell Adhesion; D015815:Cell Adhesion Molecules / Q000502:physiology*; D005109:Extracellular Matrix / Q000502:physiology; D006801:Humans; D016023:Integrins / Q000502:physiology; D052177:Kidney Diseases, Cystic / Q000473:pathology / Q000503:physiopathology*; D007684:Kidney Tubules / Q000502:physiology* / Q000503:physiopathology; D051379:Mice; D018345:Mice, Knockout; D008840:Microfilament Proteins / Q000172:deficiency / Q000502:physiology; D016891:Polycystic Kidney, Autosomal Dominant / Q000235:genetics / Q000503:physiopathology; D011506:Proteins / Q000235:genetics / Q000502:physiology; D050396:TRPP Cation Channels; D000070876:Tensins
chemical_list: D015815:Cell Adhesion Molecules; D016023:Integrins; D008840:Microfilament Proteins; D011506:Proteins; D050396:TRPP Cation Channels; D000070876:Tensins