Document Detail

Hepatocystin is not secreted in cyst fluid of hepatocystin mutant polycystic liver patients.
MedLine Citation:
PMID:  18419150     Owner:  NLM     Status:  MEDLINE    
Autosomal dominant polycystic liver disease (PCLD) is characterized by multiple liver cysts and is caused by mutations in PRKCSH (hepatocystin). Mechanisms of cystogenesis are unknown, but previous studies have shown that hepatocystin is secreted in vitro. The goal of this study was to determine the fate of hepatocystin in vivo. Using immunoprecipitation, we determined that mutant hepatocystin is secreted from both apical and basolateral cell surface of MDCK cells stably transfected with mutant hepatocystin. Analysis of 60 cyst fluid samples from polycystic livers using Western blot, MALDI-TOF MS or nLC-MS/MS did not detect hepatocystin in liver cyst fluid. We did identify 163 ubiquitous serum proteins. No paracrine or autocrine factors were recognized. Although cyst fluids vary greatly in protein concentration, a PCLD specific protein pattern was not established. In conclusion, hepatocystin is not secreted in PCLD liver cyst fluid, suggesting that mutant hepatocystin is either not produced or degraded intracellularly. PCLD cysts develop from intralobular bile ductules and cyst fluid mainly contains common serum proteins comparable to that of other polycystic diseases.
Esmé Waanders; Anke L L Lameris; Huub J M Op den Camp; Wendy Pluk; Jolein Gloerich; Simon P Strijk; Joost P H Drenth
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-04-18
Journal Detail:
Title:  Journal of proteome research     Volume:  7     ISSN:  1535-3893     ISO Abbreviation:  J. Proteome Res.     Publication Date:  2008 Jun 
Date Detail:
Created Date:  2008-06-09     Completed Date:  2008-08-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101128775     Medline TA:  J Proteome Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2490-5     Citation Subset:  IM    
Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Center, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
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MeSH Terms
Blood Proteins / analysis,  metabolism
Cell Line
Cyst Fluid / chemistry,  metabolism*
Cysts / genetics,  metabolism*
Electrophoresis, Polyacrylamide Gel
Glucosidases / genetics,  metabolism,  secretion*
Intracellular Signaling Peptides and Proteins / genetics,  metabolism,  secretion*
Liver Diseases / genetics,  metabolism*
Serum Albumin / analysis,  metabolism
Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
Tandem Mass Spectrometry
Reg. No./Substance:
0/Blood Proteins; 0/Intracellular Signaling Peptides and Proteins; 0/PRKCSH protein, human; 0/Serum Albumin; EC 3.2.1.-/Glucosidases

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