pixHep UCD (Urea Cycle Disorder) Models (ASS1, and OTC)

pixHep iPSC-derived hepatocyte models capturing key enzymatic defects in the urea cycle (ASS1 and OTC).

CRISPR-engineered, cryopreserved vials (1 × 10⁶ viable cells per vial) available as ASS1 (G390R) or OTC (D175V) mutant lines
Reproduce reduced ammonia detoxification, impaired enzyme expression, and decreased urea output
Ideal for evaluating small molecules, gene therapies, and enzyme restoration strategies targeting UCD

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Our UCD portfolio includes OTC D175V-mutant and ASS1-knockout hepatocytes, each displaying impaired urea cycle function and measurable metabolic imbalance.

These models enable direct testing of gene therapies, enzyme replacement, and metabolic modulators in a fully human system.

Reduced enzyme activity confirmed via metabolic assays.

Elevated ammonia and altered metabolite profiles.

Isogenic controls for genotype–phenotype matching.

CRISPR customisation for additional UCD variants.

Technical Data & Functional Validation

Functional Urea Cycle Pathway

Protein expression levels of the five urea cycle enzymes in liver carcinoma HepG2 cells and pixHeps. Protein data were normalized to b-actin and are presented as mean±SEM of n=3 biological replicates

Successful OTCD iPSC Differentiation

mRNA expression levels of the hepatocyte maturity markers albumin (ALB), alpha1-antitrypsin (A1AT), and hepatocyte nuclear factor 4A (HNF4A) in wild-type (WT) and OTCD pixHep (right). mRNA data were normalized to PPIA and are presented as mean±SEM of n=2 biological replicates. Magnification: 10x.

Decreased OTC Expression

Protein expression levels of OTC in wild-type (WT) and CRISPR-derived OTCD pixHep.

Decreased ASS1 Expression

Protein expression levels of ASS1 in wild-type (WT) and CRISPR-derived ASS1 pixHep.

Modeling Urea cycle disorders through CRISPR-derived OTCD pixHep

Case Study

October 18, 2025

Modeling Urea cycle disorders through CRISPR-derived OTCD pixHep

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Our pixCell portfolio seamlessly integrates into custom data generation projects—from functional assays (pixCellServices) to phenomic analysis (pixCellPaint)—delivering ready-to-use data at your fingertips.

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