A Phase 1/2 Trial of SP-101 for the Treatment of Cystic Fibrosis (CF)

Inclusion Criteria:

1. Males or females, age 18 to 65 years at Screening Visit, inclusive

2. Diagnosis of CF

3. ppFEV1 value between 50-100% (inclusive)

4. Resting oxygen saturation ≥94% on room air by pulse oximetry 5 . Clinically stableCF disease as assessed by the Investigator and not requiring any new class ofinterventional treatment within the last 3 months prior to Screening

Exclusion Criteria:

1. Any change in established pulmonary treatment (including antibiotics) within 28 daysprior to Screening Visit. However, inhaled beta-agonists can be included within 2weeks prior to Screening Visit.

2. Clinically significant episode of hemoptysis (>50 mL or ¼ cup or 10 teaspoons perday) within 12 weeks prior to dosing with study drug on Day 1

3. Lung infection with Mycobacterium abscessus associated with a more rapid decline inpulmonary status

4. Currently receiving treatment for active lung infection with Burkholderiacenocepacia or Burkholderia dolosa

5. History of solid organ or hematological transplantation

6. History of clinically significant cirrhosis with or without portal hypertension

7. History of pulmonary hypertension

8. History of cardiotoxicity, a history of known coronary artery disease, and/orexisting cardiomyopathy

9. Current active fungal infection (not just a positive culture), acute blood, lung, orbladder infection, clinically significant hepatic or renal dysfunction, and/or viralinfection (including human immunodeficiency virus or hepatitis virus B or C)requiring the initiation of new therapy within 30 days prior to Screening

10. History of allergic bronchopulmonary aspergillosis (ABPA)

11. Uncontrolled diabetes mellitus, as evidenced by hemoglobin A1c >9% at Screening

12. Clinically significant laboratory abnormalities at Screening

13. Subjects with any medical condition or abnormal laboratory result that, in theopinion of the Investigator, will interfere with the safe completion of the study

14. Subjects who received any investigational products within 30 days (or 5 therapeutichalf-lives, whichever is longer) prior to Screening

15. Subjects who have previously received any gene therapy agent

16. Subjects with known sensitivity to SP-101, doxorubicin or its excipients