Denali Therapeutics

Denali Therapeutics works on 6 rare diseases tracked on Trial Friend, including Amyotrophic Lateral Sclerosis, Glycogen Storage Disease Type II, Hunter Syndrome and 3 more, with 4 recruiting clinical trials and 1 FDA-approved rare disease drug.

Denali Therapeutics develops therapies for neurodegenerative and lysosomal storage diseases using its Transport Vehicle (TV) platform to cross the blood-brain barrier. Avlayah (tividenofusp alfa) received FDA accelerated approval in March 2026 for neurologic manifestations of Hunter syndrome (MPS II). DNL952 (ETV:GAA) for Pompe disease is in Phase 1 development.

Type
Rare Disease Specialist
Ticker
DNLI
Headquarters
South San Francisco, United States
Founded
2015
4
Active Rare Disease Trials
1
Approved Rare Disease Drugs
6
Rare Diseases in Portfolio
11
Years Active

Focus areas at Denali Therapeutics

As a rare disease specialist, Denali Therapeutics has active clinical trial programs and drug development efforts across 6 rare diseases, including Amyotrophic Lateral Sclerosis, Glycogen Storage Disease Type II, Hunter Syndrome, Mucopolysaccharidosis Type I, Sanfilippo Syndrome, and 1 additional rare conditions. These programs may span orphan drug designation, novel therapeutic mechanisms, and precision medicine approaches targeting the underlying causes of each disease.

The clinical trials section below shows all active and recruiting studies sponsored by Denali Therapeutics, sourced live from ClinicalTrials.gov. Each trial includes its current recruitment status, study phase (Phase 1 through Phase 4), conditions under investigation, and the number of active trial sites. The FDA-approved drugs section lists treatments that have received U.S. Food and Drug Administration approval, with brand names, generic names, approval dates, and matched rare disease indications from the openFDA database.

Denali Therapeutics is headquartered in South San Francisco, United States, founded in 2015, publicly traded under the ticker symbol DNLI. The company dedicates its research and development resources exclusively to rare and orphan diseases, working to bring innovative treatments to patients with conditions that have historically had limited or no treatment options.

Denali Therapeutics Drug Pipeline

Denali Therapeutics has 4 active clinical trials across 3 development stages, with 4 currently recruiting participants. Clinical trials advance through phases: Phase 1 tests safety in a small group, Phase 2 evaluates effectiveness and side effects, Phase 3 confirms benefit in a larger population, and Phase 4 monitors long-term safety after FDA approval.

Note: This pipeline includes all of Denali Therapeutics's active interventional trials, not only those targeting rare diseases. We show the full pipeline because a company's broader research activity, therapeutic expertise, and development infrastructure directly shape its ability to advance rare disease programs. A strong overall pipeline often signals deeper clinical operations, faster enrollment capabilities, and greater commitment to bringing new treatments to patients.

Understand Denali Therapeutics's pipeline
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1
Early Phase / Phase 11 trial
2
Phase 22 trials
Mucopolysaccharidosis Type IIIA
Recruiting
Mucopolysaccharidosis II
Recruiting
1
Phase 31 trial
Mucopolysaccharidosis II
Recruiting

Denali Therapeutics Clinical Trials (4)

Active and recruiting clinical trials sponsored by Denali Therapeutics, sourced live from ClinicalTrials.gov. Each trial card shows the study phase, current recruitment status, conditions under investigation, study locations, eligibility criteria, and a direct link to the full ClinicalTrials.gov record. You can also download a one-page PDF summary to share with your doctor.

Note: Recruitment statuses on ClinicalTrials.gov may not immediately reflect recent FDA decisions, sponsor announcements, or enrollment changes. Always confirm a trial's current status directly with the study coordinator.

Ask about Denali Therapeutics's trials
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ACTIVE NOT RECRUITINGPHASE2, PHASE3Recently updatedNCT05371613

A Study to Determine the Efficacy and Safety of Tividenofusp Alfa (DNL310) vs Idursulfase in Pediatric and Young Adult Participants With Neuronopathic (nMPS II) or Non-Neuronopathic Mucopolysaccharidosis Type II (nnMPS II)

Intervention: tividenofusp alfa, idursulfase

Mucopolysaccharidosis II

This is a Phase 2/3, multiregional, two-arm, double-blind, randomized, active (standard-of-care)-controlled study of the efficacy and safety of tividenofusp alfa (DNL310), an investigational central nervous system (CNS)-penetrant enzyme-replacement therapy (ERT) for mucopolysaccharidosis type II (MPS II). Participants may also qualify to enter an open-label treatment phase with DNL310 or idursulfase based on pre-specified criteria.

Ages 2 Years - 25 Years30 locations
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RECRUITINGPHASE1Recently updatedNCT07354724

A Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of DNL952 in Adult Participants With Late-Onset Pompe Disease

Intervention: DNL952

This is a Phase 1, multicenter, open-label study to evaluate the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of DNL952 in adult participants with late-onset Pompe disease. The principal aim of this study is to obtain safety and tolerability data across varous dose levels of DNL952 in participants with late-onset Pompe disease (LOPD).

Ages 18 Years - 75 Years4 locations
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ACTIVE NOT RECRUITINGPHASE1, PHASE2Recently updatedNCT06181136

Study of DNL126 in Pediatric Participants With Mucopolysaccharidosis Type IIIA (Sanfilippo Syndrome Type A)

Intervention: DNL126

Mucopolysaccharidosis Type IIIA

This is a multicenter, open-label, Phase 1/2 study to assess the safety, tolerability, pharmacokinetics (PK), pharmacodynamics (PD) and clinical efficacy of DNL126 in participants with Sanfilippo syndrome Type A (MPS IIIA). The core study period is 25 weeks (approximately 6 months); followed by an open-label extension (OLE), which extends through Week 97 (approximately 18 months); and a long-term extension (LTE), which extends through Week 193 (Year 4). Participants with MPS IIIA will be enrolled in two planned cohorts, and additional participants with MPS IIIA may be enrolled in three optional cohorts.

Ages 0 Years - 18 Years4 locations
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ACTIVE NOT RECRUITINGPHASE1, PHASE2Hasn't posted an update in over a yearNCT04251026

A Study of Tividenofusp Alfa (DNL310) in Pediatric Participants With Hunter Syndrome

Intervention: tividenofusp alfa

Mucopolysaccharidosis II

This is a multicenter, multiregional, open-label study to assess the safety, pharmacokinetics (PK), and pharmacodynamics (PD) of tividenofusp alfa (DNL310), an investigational central nervous system (CNS)-penetrant enzyme replacement therapy (ERT), designed to treat both the peripheral and CNS manifestations of Mucopolysaccharidosis type II (MPS II; Hunter syndrome). Participants, whose physicians feel they are deriving benefit, will have the opportunity to be reconsented into a safety extension and then an open-label extension for continued evaluation.

Ages Up to 18 Years7 locations
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Denali Therapeutics FDA-Approved Drugs (1)

Medications developed or marketed by Denali Therapeutics that have received U.S. Food and Drug Administration approval. Drug data is sourced from the openFDA database and includes brand names, generic names, approval dates, and matched rare disease indications. Where a drug treats a condition covered by Trial Friend, the disease name links directly to that disease page.

Drug NameBrand NameRare DiseasesApproval Date
TIVIDENOFUSP ALFA-EKNM
Hydrolytic Lysosomal Glycosaminoglycan-specific Enzyme [EPC]
AVLAYAH
intravenous
Mar 24, 2026

Denali Therapeutics Trial Locations

Denali Therapeutics clinical trials are running at 45 sites in 14 countries. Click any country to drill down by state, city, and individual research facility. Proximity to a trial site is one of the most important factors in deciding whether to participate.

United States
20▼
United Kingdom
5▼
Canada
4▼
Belgium
2▼
Germany
2▼
Netherlands
2▼
Spain
2▼
Turkey (Türkiye)
2▼
Argentina
1▼
Brazil
1▼
Czechia
1▼
France
1▼

Rare Disease Focus Areas (6)

Diseases targeted by Denali Therapeutics's clinical trial and drug development programs

Amyotrophic Lateral SclerosisNeurological & Neuromuscular

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease destroying motor neurons in the brain and spinal cord. This causes progressive weakness and paralysis of voluntary muscles whil...

Prevalence: About 5,000 new cases per year in the U.S.; approximately 16,000 Americans living with ALS at any given time
Glycogen Storage Disease Type IIMetabolic & Lysosomal

Glycogen Storage Disease Type II (GSD II, also called Pompe Disease) is a lysosomal storage disorder caused by deficiency of the enzyme acid alpha-glucosidase (GAA), which breaks down glycogen. This l...

Prevalence: Approximately 1 per 14,000 to 1 per 40,000 live births globally; infantile form is most common
Hunter SyndromeMetabolic & Lysosomal

Hunter syndrome is an X-linked lysosomal storage disorder caused by deficiency of iduronate-2-sulfatase enzyme, leading to accumulation of glycosaminoglycans in cells and tissues. This multisystem dis...

Prevalence: 1 in 100,000 to 150,000 males; females with disease are rare
Mucopolysaccharidosis Type IMetabolic & Lysosomal

Mucopolysaccharidosis Type I (MPS I) is a lysosomal storage disorder caused by deficiency of the enzyme alpha-L-iduronidase, which breaks down glycosaminoglycans (GAGs). This results in accumulation o...

Prevalence: Approximately 1 per 100,000 live births; Hurler form is the most common phenotype
Sanfilippo SyndromeMetabolic & Lysosomal

Sanfilippo syndrome, formally mucopolysaccharidosis type III (MPS III), is a lysosomal storage disorder in which the body cannot break down a sugar chain called heparan sulfate. It builds up mainly in...

Prevalence: Approximately 1 per 70,000 to 1 per 100,000 live births; highest prevalence in certain populations including Australia and Scandinavia
Pompe DiseaseMetabolic & Lysosomal

Pompe disease is a rare inherited lysosomal storage disorder caused by deficiency of the enzyme acid alpha-glucosidase, leading to excessive accumulation of glycogen in muscles and organs. This buildu...

Prevalence: 1 in 40,000 people (infantile form: 1 in 138,500; late-onset: 1 in 60,000)

Patient Resources

Organizations and resources related to Denali Therapeutics's rare disease focus areas

Frequently Asked Questions About Denali Therapeutics

Common questions about Denali Therapeutics's rare disease programs, clinical trials, and treatments.