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Maralixibat

Phase 3

Cholestatic Liver Disease (Except ALGS, PFIC, PBC and PSC) | Small molecule | Gastrointestinal |Mirum Pharmaceuticals, Inc.|Last Updated: Aug 11, 2026

Target and mechanism

Molecular targetSLC10A2
Target classInhibitor
ModalitySmall molecule

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMC
Total Trials1
Total Enrollment90

FDA Designations

No designations recorded

Clinical trial landscape

Maralixibat · 7 trials · 7 indications

Phase 3 3Phase 2 3Phase 1 1
NCT06553768Evaluation of Maralixibat in Pruritus Associated With General Cholestatic Liver Disease (EXPAND)Cholestatic Liver Disease (Except ALGS, PFIC, PBC and PSC)
ACTIVE NOT_RECRUITING90 Analytics
NCT04185363An Extension Study of Maralixibat in Patients With Progressive Familial Intrahepatic Cholestasis (PFIC)Progressive Familial Intrahepatic Cholestasis (PFIC)
COMPLETED84 Analytics
NCT03905330A Study to Evaluate the Efficacy and Safety of Maralixibat in Subjects With Progressive Familial Intrahepatic Cholestasis (MARCH-PFIC)Progressive Familial Intrahepatic Cholestasis (PFIC)
COMPLETED93 Analytics
PHASE3ACTIVE NOT_RECRUITING
Evaluation of Maralixibat in Pruritus Associated With General Cholestatic Liver Disease (EXPAND)
Cholestatic Liver Disease (Except ALGS, PFIC, PBC and PSC)Unlock trial analytics
PHASE3COMPLETED
An Extension Study of Maralixibat in Patients With Progressive Familial Intrahepatic Cholestasis (PFIC)
Progressive Familial Intrahepatic Cholestasis (PFIC)Unlock trial analytics
PHASE3COMPLETED
A Study to Evaluate the Efficacy and Safety of Maralixibat in Subjects With Progressive Familial Intrahepatic Cholestasis (MARCH-PFIC)
Progressive Familial Intrahepatic Cholestasis (PFIC)Unlock trial analytics

Study Endpoints

Primary Endpoints

Mean change in the ItchRO(Obs) severity score
From baseline to average of week 13 to week 20

ItchRO(Obs) severity score = Itch Reported Outcome Observer assessment severity score; scale between 0 (not itchy at all) and 4 (extremely itchy); the lower the score the better.

Mean Change From Baseline Over Time in the Average Morning ItchRO(Obs) Severity Score
From Baseline to Weeks 75-78

The Score on the ItchRo scale is a score on a 5-point scale from 0 (no itch) to 4 (very severe itch)

Mean Change in the Average Morning ItchRO(Obs) Severity Score in the Primary Cohort
MMRM model was used with inputs of the average changes from baseline (BL) to Weeks 1-6, 7-10, 11-14, 15-18, 19-22, 23-26, and other covariates. The average change from BL over Weeks 15-26 is calculated in the model and presented as a single number.

The primary efficacy endpoint is the mean change in the average morning ItchRO(Obs) severity score between baseline and Weeks 15-26, using 4-week average morning ItchRO(Obs) severity scores (Mixed Model Repeated Measures). The baseline average morning ItchRO(Obs) severity score is defined as the 4-week average morning ItchRO(Obs) severity score prior to the first dose of the study medication. ItchRo(Obs) Severity Score = Itch Reported Outcome Observer assessment severity score; scale between 0 (not itchy at all) and 4 (extremely itchy); the lower the score the better.

Frequency of Treatment-emergent Adverse Events [TEAEs]
From Baseline through to Week 13

TEAEs = Treatment-emergent Adverse Events

Mean Change in Total Serum Bilirubin Levels
From baseline to Week 26
Incidence of Treatment-Emergent Adverse Events
From informed consent through approximately 4.5 years, including 30 days after last dose.

TEAE = Treatment-emergent Adverse Event; AESI = Adverse Event of Special Interest..

Change From Baseline of Fecal Bile Acid Excretion After Dosing (Day 6 and Day 7)
Baseline, Day 7

Fecal bile acid excretion was determined by the concentration of total bile acids in stool samples over each 24-hour collection window during the lead-in and treatment periods.

Secondary Endpoints

Mean change in total sBA (serum bile acid) level
From baseline to average of week 12 and week 20
Maintenance of ItchRO(Obs) Response (Weeks 15 - 26)
Week 15 - 26
Proportion of ItchRO(Obs) Responders Over Time
Baseline to EOT
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Study Design & Arms

AllocationRANDOMIZED
MaskingQUADRUPLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
MaralixibatEXPERIMENTALParticipants will receive maralixibat oral solution 300 μg/kg orally once daily for 1 week and then twice daily for 39 weeks.
PlaceboPLACEBO_COMPARATORParticipants will receive placebo matched to maralixibat oral solution orally once daily for 1 week and then twice daily for 19 weeks. After 20 weeks, participants will receive maralixibat oral solution 300 μg/kg orally once daily for 1 week and then twice daily for 19 weeks.
Double Blind - MaralixibatEXPERIMENTALThe double-blind period comprised of 4-8 weeks of dose escalation followed by 18 - 22 weeks of stable dosing treatment, after which participants were transferred to the open-label arm.
Double Blind - PlaceboPLACEBO_COMPARATORThe double-blind period comprised of 4-8 weeks of dose escalation followed by 18 - 22 weeks of stable dosing treatment, after which participants were transferred to the open-label arm.
Open Label - MaralixibatEXPERIMENTALThe Open-Label period comprised of 4-8 weeks of dose escalation followed by 70 - 74 weeks of stable dosing treatment. During the OLE, all participants, regardless of treatment assignment in the double-blind period, received maralixibat.
Maralixibat 10mgEXPERIMENTALParticipants will receive maralixibat 10 mg liquid formulation orally QD for 7 days.
Volixibat 10mgEXPERIMENTALParticipants will receive volixibat 10 mg capsule orally QD for 7 days.
Maralixibat 20mgEXPERIMENTALParticipants will receive maralixibat 20 mg liquid formulation orally QD for 7 days.
Volixibat 20mgEXPERIMENTALParticipants will receive volixibat 20 mg capsule orally QD for 7 days.
Maralixibat 50mgEXPERIMENTALParticipants will receive maralixibat 50 mg liquid formulation orally QD for 7 days.
Maralixibat 50mg BIDEXPERIMENTALParticipants will receive maralixibat 50 mg liquid formulation orally BID for 7 days.
Maralixibat 100mgEXPERIMENTALParticipants will receive maralixibat 100 mg liquid formulation orally QD for 7 days.

Interventions

NameTypeDescription
MaralixibatDRUGMaralixibat will be provided as an oral solution along with 0.5-, 1.0-, and 3.0-mL sized dosing dispensers. During the double-blind dose escalation period (4 weeks), the study drug (maralixibat) will be administered once daily for 1 week and then twice daily (BID; morning and evening). During the double-blind stable dosing period (16 weeks), participants will be treated with 300 μg/kg BID or the maximum tolerated dose (determined during the double-blind dose-escalation period) of maralixibat. During the open-label dose escalation period (4 weeks), all participants will receive maralixibat treatment once daily for 1 week and then twice daily (BID; morning and evening). During the open-label stable dosing period (at least 16 weeks), participants will be treated with 300 μg/kg BID or the maximum tolerated dose (determined during the open-label dose-escalation period) of maralixibat.
PlaceboOTHERPlacebo matched to maralixibat will be provided as an oral solution along with 0.5-, 1.0-, and 3.0-mL sized dosing dispensers. During the double-blind dose escalation period (4 weeks), study drug will be administered once daily for 1 week and then twice daily (BID; morning and evening). During the double-blind stable dosing period (16 weeks), participants will be treated with 300 μg/kg BID or the maximum tolerated dose (determined during the double-blind dose-escalation period) of study drug. During the open-label dose escalation period (4 weeks), all participants will receive maralixibat treatment once daily for 1 week and then twice daily (BID; morning and evening). During the open-label stable dosing period (16 weeks), participants will be treated with 300 μg/kg BID or the maximum tolerated dose (determined during the open-label dose-escalation period) of maralixibat.
VolixibatDRUGParticipants will receive volixibat in 10 mg and 20 mg doses.
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Eligibility Criteria

Age Range6 Months to N/A
SexALL
Healthy VolunteersNo
Study Sites24

Inclusion Criteria: 1. Informed consent and assent (as applicable) 2. Age ≥6 months at time of baseline visit 3. Diagnosis of a rare cholestatic liver disease with cholestatic pruritus based on the following: 1. Chronic liver biochemical abnormalities (\>90 days) and/or pathological evidence of...

Countries:United StatesBrazilCanadaFranceGermanyItalyLebanonPolandSpainUnited KingdomArgentinaAustriaBelgiumColombiaMexicoSingaporeTurkey (Türkiye)HungaryChinaTaiwanVietnamAustralia
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Recent Changes (Last 90 Days)

LOWAug 11, 2026NCT06553768lastUpdatePostDate: changed
LOWAug 11, 2026NCT06553768lastUpdatePostDate: changed
MEDIUMJul 24, 2026NCT04185363TRIAL_REMOVED: changed
MEDIUMJul 24, 2026NCT04185363TRIAL_REMOVED: changed
MEDIUMJul 3, 2026NCT04729751TRIAL_REMOVED: changed
MEDIUMJul 3, 2026NCT04729751TRIAL_REMOVED: changed
MEDIUMJul 3, 2026NCT04729751TRIAL_REMOVED: changed

Frequently asked questions about Maralixibat

What is Maralixibat used for?

Maralixibat is an investigational small molecule being studied for cholestatic liver diseases, including progressive familial intrahepatic cholestasis (PFIC), biliary atresia, and other cholestatic conditions. It is also being evaluated in healthy volunteers. The drug is in clinical development and has not been approved by the FDA.

Who makes Maralixibat?

Maralixibat is being developed by Mirum Pharmaceuticals, Inc., a biopharmaceutical company traded on NASDAQ under the ticker MIRM. The company is conducting clinical trials to evaluate the drug's safety and efficacy in patients with cholestatic liver diseases.

What phase is Maralixibat in?

Maralixibat is in Phase 2 and Phase 3 clinical trials. Most completed studies are Phase 2, while one active trial, NCT06553768, is Phase 3. The drug is investigational and not yet approved for any indication.

What clinical trials is Maralixibat in?

Maralixibat has been studied in several trials, including NCT04168385, a long-term safety study in cholestatic liver disease; NCT04524390, evaluating response post-Kasai in biliary atresia; NCT04729751, in infants with PFIC and Alagille syndrome; and NCT06553768, the EXPAND study for pruritus in general cholestatic liver disease.

Is Maralixibat the same as MRX-800?

Yes, Maralixibat is also known as MRX-800. One clinical trial, NCT04168385, is titled 'MRX-800: A Long-Term Safety Study of Maralixibat in the Treatment of Cholestatic Liver Disease.' This alternative name may appear in clinical trial records and publications.

How does Maralixibat work?

Maralixibat is a small molecule that targets the apical sodium-dependent bile acid transporter (ASBT) in the ileum. By inhibiting this transporter, it reduces bile acid reabsorption and lowers systemic bile acid levels, which may alleviate pruritus and liver damage in cholestatic diseases.