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ORGN001

Phase 2

Molybdenum Cofactor Deficiency, Type A | Small molecule | Other |BridgeBio Pharma, Inc.|Last Updated: Oct 17, 2023

Success Probability
Approval Probability 71%
TA Base Rate26%
Adjusted LOA41%
ML RiskLOW_RISK
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Market & Valuation
rNPV $3.2B
Market Size $9.4B
Revenue Basis $1.6B
Competitors 6
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Trial Design
UNCONTROLLEDDMCBiomarker
Total Trials2
Total Enrollment13
FDA Designations
No designations recorded
Clinical Trials (2)
NCT IDTitlePhaseStatusEnrollmentVelocityDesignStartCompletionLast UpdatedSitesCountries
NCT02629393Study of ORGN001 (Formerly ALXN1101) in Neonates, Infants and Children With Molybdenum Cofactor Deficiency (MOCD) Type APHASE2 COMPLETED 5May 1, 2016Oct 1, 2022Oct 17, 202311 United States, Israel +4
NCT02047461Safety & Efficacy Study of ORGN001 (Formerly ALXN1101) in Pediatric Patients With MoCD Type A Currently Treated With rcPMPPHASE2 COMPLETED 8Apr 1, 2014Oct 1, 2022Oct 17, 20236 United States, Australia +3
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Study Endpoints
Primary Endpoints
Overall Survival
Through last observation (average of 24 months)

Patients with a confirmed diagnosis of MOCD Type A, treated with ORGN001 and still alive at last observation.

Safety of ORGN001 (Formerly ALXN1101)
Baseline to Month 24 for all patients plus additional follow-up up to Month 90

Treatment Emergent Serious Adverse Events

Secondary Endpoints
Feeding Pattern
At Month 12 visit
Pharmacokinetics (Actual Plasma Concentration) of ORGN001 (Formerly ALXN1101)
First 6 months at each dose level, where available
S-sulfocysteine (Umol/L) Normalized to Urine Creatinine (mmol/L) - Change From Baseline Over Time
Baseline to Month 24 for all patients plus additional follow-up to Month 90
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Study Design & Arms
AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT
Treatment Arms
ArmTypeDescription
ORGN001 (formerly ALXN1101)EXPERIMENTAL -
Interventions
NameTypeDescription
ORGN001 (formerly ALXN1101)DRUG -
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Eligibility Criteria
Age Range1 Day — 5 Years
SexALL
Healthy VolunteersNo
Study Sites11

Patients must meet all of the following inclusion criteria to be considered for enrollment in this study: 1. Male or female neonatal patient (1 to 28 days of age \[inclusive\] at the time of ORGN001 administration, with day 1 of age corresponding to the day of birth) or infant (29 days to \<2 years...

Countries:United StatesIsraelNorwaySpainTurkey (Türkiye)United KingdomAustraliaNetherlandsTunisia
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