Fundamental insights into autosomal dominant polycystic kidney disease from human-based cell models.
Pediatr Nephrol
; 34(10): 1697-1715, 2019 10.
Article
in En
| MEDLINE
| ID: mdl-30215095
ABSTRACT
Several animal- and human-derived models are used in autosomal dominant polycystic kidney disease (ADPKD) research to gain insight in the disease mechanism. However, a consistent correlation between animal and human ADPKD models is lacking. Therefore, established human-derived models are relevant to affirm research results and translate findings into a clinical set-up. In this review, we give an extensive overview of the existing human-based cell models. We discuss their source (urine, nephrectomy and stem cell), immortalisation procedures, genetic engineering, kidney segmental origin and characterisation with nephron segment markers. We summarise the most studied pathways and lessons learned from these different ADPKD models. Finally, we issue recommendations for the derivation of human-derived cell lines and for experimental set-ups with these cell lines.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Polycystic Kidney, Autosomal Dominant
/
Kidney
Type of study:
Guideline
/
Prognostic_studies
Limits:
Humans
Language:
En
Journal:
Pediatr Nephrol
Journal subject:
NEFROLOGIA
/
PEDIATRIA
Year:
2019
Document type:
Article
Affiliation country:
Belgium