pixHep PFIC2 (Progressive Familial Intrahepatic Cholestasis Type 2) Model

iPSC-derived hepatocytes modeling bile acid transport and cholestatic dysfunction.

Cryopreserved vials (1 × 10⁶ viable cells per vial) carrying ABCB11 D482G mutation
Show reduced bile acid export and canalicular polarization defects
Enable drug screening and mechanistic studies in cholestasis pathways via pixCellServices

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The PFIC2 pixHep™ model carries loss-of-function ABCB11 mutations, leading to impaired bile acid export and cholestatic injury.

It enables study of bile acid metabolism, inflammatory signalling, and therapeutic rescue.

Reduced/absent BSEP localisation confirmed by immunostaining.

Intracellular bile acid accumulation measured by LC–MS.

Compatible with drug, gene, or cell therapy evaluation.

Isogenic controls for direct comparison.

Technical Data & Functional Validation

ABCB11 D482G Genotypic Validation

Sanger sequencing showing wild-type (top sequence) and mutated iPSCs (bottom sequence) carrying the D482G mutation (GAT>GTT) in the ABCB11 gene. The codon change is highlighted with yellow (left). mRNA expression levels of the key pluripotency markers NANOG, SOX2, and OCT4 in wild-type (WT) and CRISPR-derived ABCB11 iPSCs (PFIC2). mRNA data were normalized to GAPDH and are presented as mean±SEM of n=3 biological replicates.

Successful Differentiation to pixHep Hepatocytes

Representative images demonstrating the characteristic hepatocyte cobblestone morphology in wild-type (WT)(left) and PFIC2 pixHeps (middle).mRNA expression levels of the hepatocyte maturity markers albumin (ALB), alpha-1-antitrypsin (A1AT), and hepatocyte nuclear factor 4A (HNF4A) in wild-type iPSCs (WT) and PFIC2 pixHeps. mRNA data were normalized to PPIA and are presented as mean±SEM of n=2 biological replicates.

Reduced ABCB11 mRNA and Protein Expression

mRNA expression levels of ABCB11 in wild-type (WT) and PFIC2 pixHeps cultured in transwell culture system (left). Protein expression levels of ABCB11 in transwell-cultured WT iPSCs and PFIC2 pixHeps in comparison to primary human hepatocytes (PHH) (middle). mRNA data were normalized to 18S rRNA, and protein data to b-actin. All data are presented as mean±SEM of n=3-4 biological replicates.

Reduced Bile Acid Transport

Taurocholic acid (TCA) quantifcation in the upper and lower transwell compartments of WT and PFIC2 pixHeps following 48 hours of TCA addition (10 µM) to the lower compartment, indicating dysfunctional ABCB11 activity in the PFIC2 cells. Data are presented as mean±SEM of n=4 biological replicate.

Modeling progressive familial intrahepatic cholestasis type 2 through CRISPR-derived PFIC2 pixHep

Case Study

October 16, 2025

Modeling progressive familial intrahepatic cholestasis type 2 through CRISPR-derived PFIC2 pixHep

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