DefiniGEN provide disease modelled MODY3 diabetes human pancreatic cells which are highly functional CRISPR gene-edited human pancreatic cells. Neonatal diabetes mellitus (NDM) is a rare but potentially devastating metabolic disorder characterized by hyperglycemia combined with low levels of insulin. The neonatal diabetes cell products display the disease phenotype in combination with general function comparable to human primary pancreatic islets. These cell products can offer disease modelling and drug discovery researchers unprecedented tool for elucidating the underlying mechanisms of this key form of monogenic diabetes.
- Highly standardized cell product containing >97% human pancreatic cells with consistent performance and biologically relevant data
- Disease circuit verified confirmed mutation in the HNF1-alpha transcription factor gene – insertion of C at codon 872 producing a frameshift mutation
- Phenotypic analysis of the cells using GSIS assays demonstrates that the insulin response to glucose challenge is dysfunctional in these disease modelled cell products in contrast to DefiniGEN’s isogenic wild-type control pancreatic cell products.
Def-PANC MODY3 Cell Morphology
In contrast to Def-PANC WT cells which display normal tightly packed pancreatic cell morphology. Def-PANC MODY3 cells display aberrant morphology expected from developmental retardation caused by the HNF1a mutation.
Disease circuit verification
MODY3 pancreatic disease model with mutation in HNF1-alpha transcription factor gene – insertion of C at codon 872 producing frameshift mutation – isogenic control available.
GLUT + PKLR Expression Analysis
The expected down regulation of pancreatic cell function gene sets is observed in Def-PANC MODY3 cells in both the disease model variants.
Pancreatic Key Marker Analysis
The expression of crucial pancreatic genes in sequentially reduced in dsiease modelled Def-PANC cells.