UNLOCKED: A Phase 2, Open-label Trial With KB195 in Subjects With a Urea Cycle Disorder

Inclusion Criteria

• •Signed informed consent and willing to comply with protocol-specified procedures.
• •Has any confirmed UCD other than N-acetyl glutamatesynthase (NAGS) deficiency.
• •Is male or female, 12 to 70 years of age (inclusive)
• •If ≥ 18 years old, has a BMI ≥20.0 and \< 40.0 kg/m2. If \< 18 years old, has a BMI between 5th percentile and 95th percentile and weight greater than 5th percentile according to age, sex and regionally appropriate growth chart
• •Has evidence of poorly controlled disease on the current standard of care (SOC)
• •If NBT is part of SOC, is on a stable dose and regimen for at least 4 weeks before Screening and the dose is expected to remain stable during the study
• •Is willing to maintain a stable diet throughout the course of study and is willing to continue usual exercise routine.
• •If taking probiotics or prebiotics, is on a stable dose regimen for at least 4 weeks before Screening and the dose and regimen are expected to remain stable during the study
• •Has a negative urine screen for drugs of abuse at Screening
• •If male or female of child bearing potential, agree with use effective method of contraception for the duration of the study and 90 days after last dose of study product