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Pancreatic KCNJ11

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info@definigen.com
UK +44 (0)1223 497106
USA 617-674-3260

DefiniGEN provide disease modelled neonatal 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 KCNJ11 gene encoding Kir6.2 subunit of potassium channels – CAT>CGT at codon 201
  • 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.

Pancreatic Cell Morphology

 

                Def-PANC WT                 

  Def-PANC Neonatal

CRISPR-modified KCJN11 pancreatic cells display normal morphology in monolayer culture

 

 

 

Figure 1. Morphology of pancreatic cells grown as a monolayer

 

Disease Circuit Verification

 

Neonatal Diabetes disease model with confirmed mutation in KCNJ11 gene encoding Kir6.2 subunit of potassium channels – CAT>CGT at codon 201 – isogenic control available

 

Sanger sequencing confirmation of a single base mutation at codon 201 of the KCNJ11 gene in KCJN11 pancreatic cells

 

Figure 2. Sanger sequencing showing heterozygous KCNJ11 mutation with single base change (CAT>CGT) at codon 201 in the gene encoding for the ATP-sensitive potassium channel subunit Kir6.2

Glucose Stimulated Insulin Secretion Assay

 

KCJN11-mutated pancreatic cells display a dysfunctional GSIS response at low and high glucose conentrations when compared to WT controls

 

Figure 3. Def-PANC WT isogenic control shows expected glucose cycling GSIS response at low and high glucose concentrations. The Def-PANC Neonatal disease model shows dysfunction in its glucose responsive insulin production in contrast to the isogenic WT control.  Genetic information and phenotypic GSIS response confirms that Def-PANC Neonatal cells are an effective model of Neonatal Diabetes.

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info@definigen.com