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CRISPR Gene Editing of ANGPTL3 Achieves Up to 80% Lipid Target Reduction in Phase 1 Cardiovascular Trial

A phase 1 dose-escalation trial of CTX310, a CRISPR-Cas9 therapy targeting hepatic ANGPTL3 to induce permanent loss-of-function mutations, showed dose-dependent ANGPTL3 reductions of up to 80% in 15 participants with uncontrolled dyslipidemia on maximally tolerated lipid-lowering therapy. No dose-limiting toxicities were observed, though one sudden death occurred 179 days after the lowest dose. This first-in-class approach could provide a one-time treatment for familial hypercholesterolemia and severe dyslipidemia, with implications for laboratory lipid panel monitoring protocols.

The original study

Phase 1 Trial of CRISPR-Cas9 Gene Editing Targeting ANGPTL3.

Authors
Laffin LJ, Nicholls SJ, Scott RS, Clifton PM, Baker J, Sarraju A, et al.
Journal
The New England journal of medicine
Type
Clinical Trial, Phase I, Journal Article, Multicenter Study
PMID
41211945
Read the original study →

Original abstract

BACKGROUND: Angiopoietin-like protein 3 (ANGPTL3) inhibits lipoprotein and endothelial lipases. ANGPTL3 loss-of-function genetic variants are associated with decreased levels of low-density lipoprotein cholesterol and triglycerides and a decreased lifetime risk of atherosclerotic cardiovascular disease. METHODS: We conducted an ascending-dose phase 1 trial to assess the safety and efficacy of CTX310, a lipid-nanoparticle-encapsulated clustered regularly interspaced short palindromic repeats-Cas9 endonuclease (CRISPR-Cas9) messenger RNA (mRNA) and guide RNA targeting hepatic ANGPTL3 to induce a loss-of-function mutation. Adults who had uncontrolled hypercholesterolemia, hypertriglyceridemia, or mixed dyslipidemia and were receiving maximally tolerated lipid-lowering therapy received a single intravenous dose of CTX310 (0.1, 0.3, 0.6, 0.7, or 0.8 mg per kilogram of body weight). The primary end point was adverse events, including dose-limiting toxic effects. RESULTS: A total of 15 participants received CTX310 and had at least 60 days of follow-up. No dose-limiting toxic effects related to CTX310 occurred. Serious adverse events occurred in two participants (13%): one participant had a spinal disk herniation, and the other died suddenly 179 days after treatment with the 0.1-mg-per-kilogram dose. Infusion-related reactions were reported in three participants (20%), and one participant (7%) who had elevated levels of aminotransferases at baseline had a transient elevation in aminotransferases to between three times and five times as high as those at baseline, peaking on day 4 and returning to baseline by day 14. The mean percent change in ANGPTL3 level was 9.6% (range, -21.8 to 71.2) with the dose of 0.1 mg per kilogram, 9.4% (range, -25.0 to 63.9) with 0.3 mg per kilogram, -32.7% (range, -51.4 to -19.4) with 0.6 mg per kilogram, -79.7% (range, -86.8 to -72.5) with 0.7 mg per kilogram, and -73.2% (range, -89.0 to -66.9) with 0.8 mg per kilogram. CONCLUSIONS: Editing of ANGPTL3 was associated with few adverse events and resulted in reductions from baseline in ANGPTL3 levels. (Funded by CRISPR Therapeutics; Australia New Zealand Clinical Trials Registry number, ACTRN12623000809639.).