Study to Evaluate the Efficacy and Safety of BBP-418 (Ribitol) in Patients With Limb Girdle Muscular Dystrophy 2I (LGMD2I)

PHASE3Active, not recruitingINTERVENTIONAL
Enrollment

81

Participants

Timeline

Start Date

May 31, 2023

Primary Completion Date

July 31, 2027

Study Completion Date

July 31, 2027

Conditions
Limb-Girdle Muscular Dystrophy Type 2I (LGMD2I)
Interventions
DRUG

BBP-418 (ribitol)

The BBP-418 drug product is provided as Granules for Oral solution consisting of BBP-418 drug substance and silicon dioxide in a multilaminate sachet with a foil barrier. Silicon dioxide is generally regarded as safe and listed in the FDA IIAD. Silicon dioxide is a compendial excipient and is commonly used in pharmaceutical dosage forms. The BBP-418 Granules for Oral Solution are provided in sachets.The BBP-418 Granules for Oral Solution are reconstituted in water for oral administration.

OTHER

Placebo

A 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.

Trial Locations (21)

19104

The Children's Hospital of Philadelphia, Philadelphia

University of Pennsylvania, Philadelphia

21205

Kennedy Krieger Institute, Baltimore

23455

Children's Hospital of the Kings Daughters, Norfolk

32610

University of Florida, Gainsville

52242

University of Iowa, Iowa City

55455

University of Minnesota, Twin Cities, Minneapolis

63110

Washington University School of Medicine, St Louis

66160

University of Kansas Medical Center, Kansas City

72202

Arkansas Children's Hospital, Little Rock

80045

University of Colorado Anschutz Medical Campus, Aurora

92697

University of California Irvine, Irvine

97239

Oregon Health & Science University (OHSU) - Neurology Clinic - South Waterfront, Portland

Unknown

Royal Brisbane and Women's Hospital,, Brisbane

Rigshospitalet, Neuromuscular Clinic and Research Unit, Copenhagen

Charité Universitätsmedizin Berlin and Max Delbrück Center, Berlin

IRCCS Ca' Granda Ospedale, Milan

Leiden University Medical Center, Leiden

Universitetssykehuset Nord-Norge, Department of Neurology, Tromsø

Great Ormond Street Hospital for Children, London

International Centre for Life, Newcastle upon Tyne

Sponsors
All Listed Sponsors
lead

ML Bio Solutions, Inc.

INDUSTRY