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DNL126

Phase 1

Mucopolysaccharidosis Type IIIA | Small molecule | Rare Disease |Denali Therapeutics Inc.|Last Updated: Jul 13, 2026

Success Probability

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Market & Valuation

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Trial Design

CONTROLLEDDMC
Total Trials1
Total Enrollment20

FDA Designations

ORPHAN_DRUGFAST_TRACKACCELERATED_APPROVAL

Clinical trial landscape

DNL126 · 1 trial · 1 indication

Phase 1 1
NCT06181136Study of DNL126 in Pediatric Participants With Mucopolysaccharidosis Type IIIA (Sanfilippo Syndrome Type A)Mucopolysaccharidosis Type IIIA
ACTIVE NOT_RECRUITING20 Analytics
PHASE1ACTIVE NOT_RECRUITING
Study of DNL126 in Pediatric Participants With Mucopolysaccharidosis Type IIIA (Sanfilippo Syndrome Type A)
Mucopolysaccharidosis Type IIIAUnlock trial analytics

Study Endpoints

Primary Endpoints

Change from baseline in the natural logarithm of cerebrospinal fluid (CSF) heparan sulfate (HS) concentration
49 weeks

Secondary Endpoints

Change from baseline in the natural logarithm of urine HS (normalized to creatinine) concentration
49 weeks
Change from baseline in liver volume multiples of normal
49 weeks
Change from baseline in the natural logarithm of serum neurofilament light chain (NfL) concentration
73 weeks
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Study Design & Arms

AllocationNON_RANDOMIZED
MaskingNONE
ModelSEQUENTIAL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Cohort A1EXPERIMENTALParticipants with MPS IIIA
Cohort A2EXPERIMENTALParticipants with MPS IIIA
Cohort A3EXPERIMENTALParticipants with MPS IIIA
Cohort B1EXPERIMENTALParticipants with MPS IIIA
Cohort B2EXPERIMENTALParticipants with MPS IIIA

Interventions

NameTypeDescription
DNL126DRUGintravenous repeating dose
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Eligibility Criteria

Age Range0 Years to 18 Years
SexALL
Healthy VolunteersNo
Study Sites4

Key Inclusion Criteria: * Confirmed diagnosis of MPS IIIA * For Cohort A2: No more than 1 participant may have predictors of a slow-progressing phenotype * For Cohort A3: Approximately 2 participants will have predictors of the slow-progressing phenotype * For Cohort B1: Have a severe phenotype bas...

Countries:United States
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Recent Changes (Last 90 Days)

LOWJul 13, 2026NCT06181136lastUpdatePostDate: changed
LOWJul 13, 2026NCT06181136lastUpdatePostDate: changed

Frequently asked questions about DNL126

What is DNL126 used for?

DNL126 is an investigational small molecule being developed for the treatment of Mucopolysaccharidosis Type IIIA, also known as Sanfilippo Syndrome Type A. It is currently in Phase 1 clinical development for this rare disease indication.

Who is developing DNL126?

DNL126 is being developed by Denali Therapeutics Inc., a biopharmaceutical company traded on the NASDAQ under the ticker symbol DNLI. The company is conducting clinical trials of DNL126 in the United States.

What phase is DNL126 in?

DNL126 is in Phase 1 clinical development. It is an investigational drug and has not been approved by the FDA. The drug has received Orphan Drug, Fast Track, and Accelerated Approval designations from the FDA.

What clinical trials is DNL126 in?

DNL126 is being studied in a Phase 1 clinical trial with the identifier NCT06181136. This trial is titled 'Study of DNL126 in Pediatric Participants With Mucopolysaccharidosis Type IIIA (Sanfilippo Syndrome Type A)' and is active but not recruiting participants.

Is DNL126 the same as Sanfilippo Syndrome Type A treatment?

DNL126 is an investigational treatment specifically for Mucopolysaccharidosis Type IIIA, which is also known as Sanfilippo Syndrome Type A. It is being studied in pediatric participants with this condition.