MODY3 Diabetes Pancreatic Cells
DefiniGEN MODY3 Diabetes Disease Modelled Pancreatic Cells
DefiniGEN’s MODY3 diabetes human pancreatic cells display a mutation in the HNF1-alpha transcription factor gene specifically an insertion of C at codon 872 producing a frameshift mutation. MODY3 (also known as HNF1A-MODY) is caused by mutations in the HNF1-alpha gene which is a key regulatory transcription factor controlling the downstream regulation of multiple genes involved in the differentiation of beta cells. Gene expression analysis of key gene sets show the expected down regulation of genes including GLUT and PKLR as well as the reduction of insulin gene expression. Furthermore the developmental retardation in the disease modelled cell manifests as aberrant morphology in contrast to DefiniGEN’s isogenic wild-type control pancreatic cell products which display normal pancreatic cell morphology. These cell products can offer disease modelling and drug discovery researchers unprecedented tool for elucidating the underlying mechanisms of this MODY. Our custom services can also engineer bespoke mutations for additional forms of the eleven know types of MODY.
- Product ID:
- Def-PANC MODY
- Cell Number:
- 10 million viable cells
- Monogenic Diabetes (MODY3)
- HNF1-alpha (heterozygous)
- Disease Modelling and Drug Discovery
Def-PANC MODY3 Cell Morphology
Genetic information and phenotypic response confirm Def-PANC MODY3 cells are an effective model of Monogenic Diabetes.
Figure 1. Def-PANC MODY3 cells display aberrant morphology in both monolayer and islet culture.
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.
Figure 2. P291 in exon 4. Insertion of C Heterozygous line available.
GLUT + PKLR Expression Analysis
Figure 3. Heterozygous and homozygous Def-PANC MODY3 cells show progressive gene change effects in key HNF1a gene regulated genes including PKLR and GLUT-2.
Pancreatic Key Marker Analysis
Figure 4. Key marker analysis by qPCR shows reduction in key pancreatic cell markers in the Def-PANC disease models.
Brochure: Coming Soon
Scientific Poster: Poster_Definigen_ISSCR 2016
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