pixHep A1ATD (Alpha-1 Antitrypsin Deficiency) Models

pixHep iPSC-derived hepatocyte models representing patient-derived and CRISPR-engineered genotypes of SERPINA1-E342K (PiZZ and PiMZ).

Cryopreserved vials (1 × 10⁶ viable cells per vial) available as patient-derived PiZZ, CRISPR PiZZ (homozygous), and CRISPR PiMZ (heterozygous) lines
Reproduce A1AT misfolding, aggregation, and intracellular inclusion formation
Suitable for proteostasis and autophagy pathway analysis, therapeutic rescue screening, and comparative mechanism-of-action studies

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Our A1ATD pixHep™ model carries the SERPINA1 Z mutation, producing misfolded A1AT protein and accumulation within hepatocytes.

It is ideal for studying ER stress, protein aggregation, and therapeutic rescue strategies.

Validated A1AT misfolding and accumulation phenotype.

Quantifiable ER stress and apoptosis markers.

Compatible with gene therapy and proteostasis-targeting compounds.

Available with isogenic wild-type controls.

Technical Data & Functional Validation

CRISPR Derived Model

The area highlighted in yellow shows homozygous E342K mutation (GAG > AAG) in the clone sequence.

Phenotypic Validation of Polymeric A1AT (ZZ)

Immunostaining with polymer-specific A1AT antibody shows levels of polymeric A1AT in ZZ-hepatocytes across the central wells of a 96 well plate. Representative images of 3 independent experiments.

Quantification of the intracellular levels of polymeric A1AT in in ZZ-hepatocytes across the central wells of a 96 well plate relative to the average of the plate set as 1. Results expressed as average from n=3 independent experiments.

Intracellular of polymeric A1AT in ZZ-hepatocytes

Intracellular accumulation of polymeric A1AT in ZZ-hepatocytes in response to two compounds that induce autophagy following immunostaining with polymer-specific A1AT antibody.

Decrease of intracellular polymeric A1ATD

Quantification of the intracellular levels of polymeric A1AT in cells treated with different concentrations of compounds that induce autophagy.

Patient-derived A1AT Accumulation

**Above:** Intracellular accumulation of polymeric A1AT in pixlbio patient-derived A1ATD hepatocytes across the central wells of a 96 well plate following immunostaining with polymer-specific A1AT antibody. Representative images of 3 independent experiments. **Below:** Quantification of the intracellular levels of polymeric A1AT in pixlbio A1ATD hepatocytes across the central wells of a 96 well plate (n=3).

Donor dependent A1AT Acumulation

Patient-derived pixHeps respond to Carbamazepine

**Above:** Quantification of intracellular polymeric A1AT in 3 different pixlbio patient-derived ZZ-A1AT hepatocytes in response to carbamazepine (CBZ) (n=3). Results expressed relative to vehicle set as 100. **Below:** Representative images of the polymeric staining.

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