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BBP-418

Phase 3

Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I) | Small molecule | Neurology |BridgeBio Pharma, Inc.|Last Updated: Jul 21, 2026

Target and mechanism

ModalitySmall molecule

Also known as BBP-418 (ribitol)

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMCBiomarker
Total Trials2
Total Enrollment188

FDA Designations

ACCELERATED_APPROVALRARE_PEDIATRIC_DISEASEPRIORITY_REVIEW

Clinical trial landscape

BBP-418 · 3 trials · 2 indications

Phase 3 2Phase 2 1
NCT07678775Open-Label Extension Study of BBP-418 (Ribitol) for LGMD2I/R9Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I)
ACTIVE NOT_RECRUITING107 Analytics
NCT05775848Study to Evaluate the Efficacy and Safety of BBP-418 (Ribitol) in Patients With Limb Girdle Muscular Dystrophy 2I (LGMD2I)Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I)
ACTIVE NOT_RECRUITING81 Analytics
PHASE3ACTIVE NOT_RECRUITING
Open-Label Extension Study of BBP-418 (Ribitol) for LGMD2I/R9
Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I)Unlock trial analytics
PHASE3ACTIVE NOT_RECRUITING
Study to Evaluate the Efficacy and Safety of BBP-418 (Ribitol) in Patients With Limb Girdle Muscular Dystrophy 2I (LGMD2I)
Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I)Unlock trial analytics

Study Endpoints

Primary Endpoints

Frequency and severity of treatment-emergent adverse events to assess long-term safety of BBP-418.
36 months
Change from baseline in North Star Assessment for LGMD2I/R9 to assess long-term efficacy of BBP-418.
36 months
Change from baseline in North Star Assessment for Limb Girdle Muscular Dystrophy following 36 months of treatment to assess efficacy of BBP-418 or placebo
36 months
Frequency and severity of treatment-emergent adverse events following 36 months of treatment to assess safety of BBP-418 or placebo
36 months
Incidence of treatment-emergent adverse events (TEAEs) that lead to dose decrease or discontinuation
60 months

Secondary Endpoints

Change from baseline in 10MWT (10-meter walk test) (m/s) for LGMD2I/R9 to assess long-term efficacy of BBP-418.
36 months
Change from baseline FVC (forced vital capacity) (percent predicted, performed in a sitting position) for LGMD2I/R9 to assess long-term efficacy of BBP-418.
36 months
Change from baseline in PUL 2.0 (performance of the upper limb) total score for LGMD2I/R9 to assess long-term efficacy of BBP-418.
36 months
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Study Design & Arms

AllocationNA
MaskingNONE
ModelSINGLE_GROUP
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
BBP-418EXPERIMENTALDrug: BBP-418. Single arm.
Placebo to Match BBP-418PLACEBO_COMPARATORThe placebo will be identical to the BBP-418 Granules for Oral Solution in appearance, packaging, labeling, and storage conditions.
Cohort 1EXPERIMENTALSubjects will receive 6 grams of BBP-418 once daily x 90 days, then 12 grams twice daily (BID, a least 8 hours apart) of BBP-418 daily until study completion.
Cohort 2EXPERIMENTALSubjects will receive 6 grams of BBP-418 twice daily (BID, at least 8 hours apart) x 90 days, then 12 grams BID of BBP-418 daily until study completion.
Cohort 3EXPERIMENTALSubjects will receive 12 grams of BBP-418 twice daily (BID, at least 8 hours apart) x 90 days, then 12 grams BID of BBP-418 daily until study completion.

Interventions

NameTypeDescription
BBP-418 (ribitol)DRUGDrug: BBP-418. Single arm. Participants who completed Study MLB-01-005 and meet eligibility criteria will receive BBP-418 (ribitol) taken orally twice daily at 9 or 12g (based on body weight measured) for up to 36 months.
PlaceboOTHERA placebo matched for similar taste and appearance was compounded at the clinical pharmacy with sucralose as a 0.35 mg/mL oral solution in purified water (USP) in a glass container. Sucralose is similar in taste to the compounded drug product.
BBP-418DRUGBBP-418 is being developed for the treatment of patients with Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I) for which no approved therapy currently exists. It targets the molecular defect at the source by supplying excess substrate to the mutant enzyme thus boosting glycosylation of muscle α-dystroglycan. The BBP-418 drug product will be packaged in sachets and provided in a carton for in-clinic and at home use.
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Eligibility Criteria

Age Range12 Years to 60 Years
SexALL
Healthy VolunteersNo
Study Sites20

Inclusion Criteria: * Completed Study MLB-01-005 on study drug through the final clinic visit (Month 36 or another qualifying end-of-study visit as determined by the Sponsor). * The participant (or parent/guardian) who signs the ICF understands the study procedures and agrees to participate in the ...

Countries:United StatesAustraliaDenmarkGermanyItalyNetherlandsNorwayUnited Kingdom
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Frequently asked questions about BBP-418

What is BBP-418 used for?

BBP-418 is an investigational small molecule being developed for Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I), a genetic muscle disorder. It is also known as ribitol. The drug is currently in clinical development and has not been approved by the FDA.

What does BBP-418 target?

The specific molecular target of BBP-418 is not disclosed in the available information. It is a small molecule being studied for its potential to treat LGMD2I, a form of muscular dystrophy. The mechanism of action has not been detailed in public clinical trial summaries.

Who is developing BBP-418?

BBP-418 is being developed by BridgeBio Pharma, Inc., a biopharmaceutical company traded on NASDAQ under the ticker BBIO. The company is conducting clinical trials to evaluate the drug's safety and efficacy in patients with LGMD2I.

What phase is BBP-418 in?

BBP-418 is in Phase 2 and Phase 3 clinical trials. A Phase 2 study (NCT04800874) is active but not recruiting, and two Phase 3 studies (NCT05775848 and NCT07678775) are also active but not recruiting. The drug is investigational and not yet FDA approved.

What clinical trials is BBP-418 in?

BBP-418 is being studied in three active trials: NCT04800874, a Phase 2 study in LGMD2I patients; NCT05775848, a Phase 3 efficacy and safety study; and NCT07678775, a Phase 3 open-label extension study. These trials are enrolling patients aged 12 and older across multiple countries.

Is BBP-418 the same as ribitol?

Yes, BBP-418 is also known as ribitol. Clinical trial records refer to the drug as BBP-418 (ribitol), and the Phase 3 study NCT05775848 explicitly evaluates BBP-418 (Ribitol) in patients with LGMD2I.