Recruiting
Phase 1/2
A Study of TSRA-196 in Adults With PiZZ Alpha-1 Antitrypsin Deficiency (AATD)
Condition(s) studied
Alpha-1 Antitrypsin Deficiency (AATD)
Investigational drug(s) / intervention(s)
TSRA-196
TSRA-196: TSRA-196 is an in-vivo genome editing product formulated in lipid nanoparticles (LNPs) for the treatment of patients with alpha-1 antitrypsin deficiency (AATD), via intravenous (IV) infusion
Study summary
This is a Phase 1/2, open-label, multi-center, dose escalation (Part 1), dose expansion (Part 2), and single repeat dose (Part 3) study to evaluate the safety, tolerability, efficacy, and PK/PD parameters of TSRA-196 in adults with the PiZZ genotype who have lung and/or liver disease associated with severe alpha-1 antitrypsin deficiency (AATD)
Eligibility
Inclusion Criteria:
* Males or females who are 18 to 70 years of age, inclusive, at the time of signing the informed consent
* Body mass index of 18 to 37 kg/m2, inclusive
* Confirmed diagnosis of AATD and PiZZ genotype
* At least one previous measure of blood total AAT level \<11 µmol/L
* Nonsmoker for at least 6 months before screening and must remain nonsmoking for the entire study duration
* Either AAT treatment-naïve or washed out of all investigational or approved treatments that modify AAT levels for 5 half-lives or at least 4 weeks, whichever is longer, before TSRA-196 administration
Parts 1A and 2A (AATD lung disease with no or minimal liver fibrosis)
* Clinically significant lung disease, defined as 1) evidence of emphysema or bronchiectasis by computed tomography or 2) DLCO \<70% of the predicted value or 3) ppFEV1 \<80%
* ppFEV1 ≥35%
* METAVIR fibrosis score F0 or F1 confirmed by liver biopsy at screening, or a liver stiffness measure by FibroScan ≤7 kPa at screening
* FIB-4 index score ≤3.25 at screening
* ALT and/or AST \<ULN at screening
Parts 1B and 2B (AATD liver disease with significant or severe liver fibrosis, with or without AATD lung disease)
* METAVIR fibrosis score F2 or F3 confirmed by liver biopsy at screening. A liver biopsy conducted within 12 months before screening is acceptable as a substitute.
* Liver stiffness measure by FibroScan \>7 and ≤15 kPa at screening
* ALT and/or AST \<2 x ULN at screening
Exclusion Criteria:
* Presence of genetic variation in SERPINA1 gene that may disrupt the function of TSRA-196, determined by screening genotyping
* History of liver disease unrelated to AATD, or history of or clinical signs of cirrhosis
* Significant lung disease not attributable to manifestations of AATD, as determined by the investigator
* History of one or more hospitalizations due to severe exacerbation of underlying lung disease during the year before screening or received IV antibiotics for treatment of a pulmonary infection within 6 months before screening
* Unstable AATD-related COPD, as determined by the investigator, or severe bronchiectasis
* Lung volume reduction surgery within 1 year before screening or plan to receive lung volume reduction surgery during the study period
* Documented chronic need for positive airway pressure therapy beyond nocturnal use
* Seropositive for human immunodeficiency virus (HIV) (HIV-1 or HIV-2)
* Seropositive for hepatitis B (hepatitis B surface antigen \[HBsAg\] or hepatitis B core antibody \[HBcAb\] positive) with detectable HBV DNA
* Hepatitis C virus (HCV) RNA positive at screening (Parts 1A and 2A), or HCV RNA positive and/or HCV antibody positive at screening (Parts 1B and 2B)
* Has received an organ transplant or is on a waiting list for an organ transplant
* Prior treatment with gene therapy using viral vectors or intended to permanently change the patient's DNA
* Any investigational products within 30 days before dosing or plan to take an investigational product before the end of study
Primary outcome measure(s)
- Part 1 (Dose Escalation): Incidence of treatment-emergent adverse events (TEAEs) and serious adverse events (SAEs) — 1 Year
- Part 2 (Dose Expansion): Proportion of participants who have serum levels of total alpha-1 antitrypsin (AAT) greater than or equal to Lower Limit of Normal (LLN) after TSRA-196 treatment — 1 Year
- Part 2 (Dose Expansion): Change in functional AAT concentrations (determined using an elastase inhibition assay) from baseline to end of study — 1 Year
- Part 3 (Single Repeated Dose): Proportion of participants who have serum levels of total alpha-1 antitrypsin (AAT) greater than or equal to Lower Limit of Normal (LLN) after a second dose of TSRA-196 — 1 Year
Trial sites (8)
| Facility | City | Region | Status |
| Clinical Study Center |
Jacksonville |
Florida |
Recruiting |
| Clinical Study Center |
Boston |
Massachusetts |
Recruiting |
| Clinical Study Center |
Rochester |
Minnesota |
Recruiting |
| Clinical Study Center |
Charleston |
South Carolina |
Recruiting |
| Clinical Study Center |
Seattle |
Washington |
Recruiting |
| Clinical Study Center |
Fitzroy |
Victoria |
Recruiting |
| Clinical Study Center |
London |
United Kingdom |
Recruiting |
| Clinical Study Center |
Nottingham |
United Kingdom |
Recruiting |