ECHS1 Knockout HAP1 Polyclonal Cells are a heterogeneous population of CRISPR/Cas9-edited HAP1 cells carrying targeted disruption of the ECHS1 gene. This polyclonal pool provides a convenient loss-of-function model for studying mitochondrial short-chain enoyl-CoA hydratase function without the need for single-cell cloning. The knockout population is supplied as live cells, validated for target gene disruption, and ready for expansion and downstream assays. As a polyclonal preparation, it preserves diverse editing events, minimizing clonal artifacts and enabling robust population-level analyses.
he HAP1 host cell line is a near-haploid human adherent line derived from KBM-7 chronic myeloid leukemia cells, carrying the BCR-ABL1 fusion oncogene. With a single copy of most chromosomes except chromosome 8, HAP1 provides a clean genetic background that unmasks knockout phenotypes efficiently. Its rapid proliferation and stable karyotype make it ideal for functional genomics, drug screening, and CRISPR-based gene perturbation studies in biomedical research.
ECHS1 encodes mitochondrial short-chain enoyl-CoA hydratase, which catalyzes the hydration of trans-2-enoyl-CoA to L-3-hydroxyacyl-CoA in the fatty acid beta-oxidation pathway. It operates downstream of ACADS and upstream of HADH, facilitating the breakdown of short-chain fatty acids and branched-chain amino acids. ECHS1 transcription is activated by PPARA and PPARGC1A and can be modulated by HIF1A. Within the mitochondrial matrix, ECHS1 interacts with HSD17B10 and ACAT1, channeling acetyl-CoA into the TCA cycle. Knockout disrupts this metabolic sequence, leading to accumulation of enoyl-CoA esters, impaired beta-oxidation, and reduced acetyl-CoA and ATP production.
In the HAP1 chronic myeloid leukemia context, loss of ECHS1 provides a defined model to examine the reliance of leukemia cells on mitochondrial fatty acid oxidation. The near-haploid genome ensures unambiguous loss of enzyme activity, allowing rigorous investigation of metabolic reprogramming in cancer. This model is particularly relevant for studying ECHS1 deficiency disorders, including Leigh syndrome and paroxysmal exercise-induced dystonia, as well as mitochondrial encephalopathies. Researchers can use these cells to assess compensatory metabolic pathways, changes in mitochondrial membrane potential, and adaptation to nutrient stress.
These polyclonal knockout cells are suitable for a range of assays, including western blotting, RT-qPCR, Seahorse metabolic flux analysis, ATP production measurements, and cell proliferation studies. They support metabolic drug testing, genetic interaction screens, and multi-omics approaches to identify metabolic vulnerabilities. The polyclonal format enables both bulk population and single-cell analyses. For further technical information or to order, please contact Ascent Research.
