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ProteomicsQuantitative Proteomic Study Unmasks Fibrinogen Pathway in Polycystic Liver Disease
(1) Background: Polycystic liver disease (PLD) is a heterogeneous group of congenital disorders characterized by bile duct dilatation and cyst development derived from cholangiocytes. Nevertheless, the cystogenesis mechanism is currently unknown and the PLD treatment is limited to liver transplantat...
ORGANISM(S): Mus musculus (Mouse) 
2022-04-04 | PXD031253 | Pride
Pld  swath  quantitative proteomics  therapeutic targets 
ProteomicsProteostasis disturbances and endoplasmic reticulum stress contribute to polycystic liver disease: new therapeutic targets.
Polycystic liver disease (PLD) is a group of genetic disorders characterized by bile duct dilatation and/or progressive development of multiple intrahepatic fluid-filled biliary cysts (>10), which are the main cause of morbidity. Current surgical and/or pharmacological therapies exert short-term and...
ORGANISM(S): Homo sapiens (Human) Rattus norvegicus (Rat) 
2020-05-25 | PXD017587 | Pride
Human and rat cholangiocytes Endoplasmic reticulum stress (er stress) 4-phenylbutyric acid (4-pba) Whole cell extracts Tims-tof pasef Polycystic liver disease (pld) Peaksq Perseus. Proteostasis 
TranscriptomicsChromosomal abnormalities in hepatic cysts point to novel polycystic liver disease genes
Autosomal dominant polycystic liver disease (ADPLD) is caused by mutations in PRKCSH, SEC63, and LRP5, while autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1, and PKD2. Liver cyst development in these disorders is explained by somatic loss-of-heterozygosity (LOH) o...
ORGANISM(S): Homo sapiens 
2016-06-30 | E-GEOD-78808 | biostudies-arrayexpress
ProteomicsTargeting UBC9-mediated Protein Hyper-SUMOylation in Cystic Cholangiocytes Halts Polycystic Liver Disease in Experimental Models
Background & Aims: polycystic liver diseases (PLDs) are genetic disorders characterized by progressive development of multiple fluid-filled biliary cysts. Most PLD-causative genes participate in protein biogenesis and/or transport. Post-translational modifications (PTMs) are implicated in protein st...
ORGANISM(S): Homo sapiens (Human) 
2020-09-29 | PXD021140 | Pride
Hepatic cystogenesis Post-translational modifications Sumoylation S-adenosylmethionine (same) Therapy. 
ProteomicsTargeting UBC9-mediated Protein Hyper-SUMOylation in Cystic Cholangiocytes Halts Polycystic Liver Disease in Experimental Models
Background & Aims: polycystic liver diseases (PLDs) are genetic disorders characterized by progressive development of multiple fluid-filled biliary cysts. Most PLD-causative genes participate in protein biogenesis and/or transport. Post-translational modifications (PTMs) are implicated in protein st...
ORGANISM(S): Homo sapiens (Human) 
2020-09-29 | PXD021139 | Pride
Hepatic cystogenesis Post-translational modifications Sumoylation S-adenosylmethionine (same) Therapy. 
ProteomicsPevonedistat inhibits aberrant protein NEDDylation in Polycystic Liver Diseases.
Background: Polycystic liver diseases (PLD) are genetic disorders characterized by progressive growth of numerous liver cysts, causing significant morbidity. Previous studies revealed genes affecting protein biogenesis and more recently, protein SUMOylation, a posttranslational modification (PTM), h...
ORGANISM(S): Homo sapiens (Human) 
2022-02-16 | PXD022571 | Pride
Hepatic cystogenesis  mln4924  pevonedistat  neddylation  therapy  nae1. 
UnknownDevelopment and Validation of a Disease-Specific Questionnaire to Assess Patient-Reported Symptoms in Polycystic Liver Disease.
Not available
S-EPMC4917464 | biostudies-literature
UnknownMedical Management of Polycystic Liver Disease: A Position Statement From the European Reference Network on Hepatological Diseases.
Not available
S-EPMC12670357 | biostudies-literature
TranscriptomicsIllumina Human Polycystic Liver Disease and Normal Biliary Stem Cell RNAseq
Background & aims: Polycystic liver disease (PLD) is an autosomal dominantly inherited disorder caused by mutations in genes such as PRKCSH and SEC63. It has been thought that cysts develop from biliary progenitor cells due to loss-of-heterozygosity (LOH), leading to aberrant proliferation or defect...
ORGANISM(S): Homo sapiens 
2015-09-30 | GSE73579 | GEO
UnknownQuestionnaire PLD-complaint-specific assessment identifies need for therapy in polycystic liver disease: A multi-centric prospective study.
Not available
S-EPMC10493353 | biostudies-literature
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