title: Differential expression of renal proteins in a rodent model of Meckel syndrome.
abstract: BACKGROUND
  Meckel syndrome (MKS) is a fatal autosomal recessive condition with prominent renal cystic pathology. Renal protein misexpression was evaluated in the Wpk rat model of human MKS3 gene disease to identify biomarkers for the staging of renal cystic progression.
  
  
  METHODS
  Misexpressed proteins were compared between early and late stages of MKS renal cystic disease using proteomic analysis (two-dimensional gel electrophoresis with LC-MS/MS identification) followed by Western blot analysis.
  
  
  RESULTS
  A proteomic analysis identified 76 proteins with statistically different, normalized abundance in at least one group. Subsequently, Western blot was used to confirm differential expression in several of these and polycystic kidney disease (PKD)-associated proteins. Galectin-1 and vimentin were identified as overexpressed proteins, which have been previously found in the jck mouse model of nephronophthisis 9. Ciliopathic PKD proteins, polycystins 1 & 2, and fibrocystin were also differentially expressed in Wpk kidney.
  
  
  CONCLUSIONS
  In the Wpk rat, misexpressed proteins were identified that were also implicated in other forms of cystic disease. Numerous proteins were either over- or underexpressed in late-stage disease. Differences in protein expression may serve as biomarkers of cystic disease and its progression.
authors.affiliation: Department of Anatomy and Cell Biology, Indiana University School of Medicine, Indianapolis, USA.
authors.forename: Stephen B
authors.initials: SB
authors.lastname: Mason
authors.forename: Xianyin
authors.initials: X
authors.lastname: Lai
authors.forename: Heather N
authors.initials: HN
authors.lastname: Ringham
authors.forename: Robert L
authors.initials: RL
authors.lastname: Bacallao
authors.forename: Peter C
authors.initials: PC
authors.lastname: Harris
authors.forename: Frank A
authors.initials: FA
authors.lastname: Witzmann
authors.forename: Vincent H
authors.initials: VH
authors.lastname: Gattone
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002925:Ciliary Motility Disorders / Q000235:genetics / Q000378:metabolism; D004195:Disease Models, Animal; D015180:Electrophoresis, Gel, Two-Dimensional; D004677:Encephalocele / Q000235:genetics / Q000378:metabolism; D000074026:Fatty Acid Binding Protein 3; D050556:Fatty Acid-Binding Proteins / Q000378:metabolism; D005260:Female; D037161:Galectins / Q000378:metabolism; D006801:Humans; D007668:Kidney / Q000378:metabolism*; D007797:Laminin / Q000378:metabolism; D008297:Male; D051379:Mice; D007690:Polycystic Kidney Diseases / Q000235:genetics / Q000378:metabolism; D020543:Proteome / Q000378:metabolism*; D040901:Proteomics; D051381:Rats; D011922:Rats, Mutant Strains; D011956:Receptors, Cell Surface / Q000378:metabolism; D050396:TRPP Cation Channels / Q000378:metabolism; D053719:Tandem Mass Spectrometry; D014746:Vimentin / Q000378:metabolism
chemical_list: D000074026:Fatty Acid Binding Protein 3; D050556:Fatty Acid-Binding Proteins; D037161:Galectins; D007797:Laminin; D020543:Proteome; D011956:Receptors, Cell Surface; D050396:TRPP Cation Channels; D014746:Vimentin; C509948:FABP3 protein, rat; C479541:PKHD1 protein, rat