Clinical trial • Phase I/II • Neurology

DNL593 for Frontotemporal dementia | Frontotemporal dementia (GRN mutation)

Phase I/II trial of DNL593 for Frontotemporal dementia | Frontotemporal dementia (GRN mutation).

Overview

Trial Therapeutic Area
Neurology
Trial Disease
Frontotemporal dementia | Frontotemporal dementia (GRN mutation)
Trial Stage
Phase I/II
Drug Modality
Peptide/protein/enzyme

Key dates

Initial CTIS Submission Date
08-04-2024
First CTIS Authorization Date
15-05-2024

Trial design

Randomised, open-label, placebo (dnl593 placebo, sterile lyophilisate in single-dose glass vials). no dose or schedule for placebo specified in provided data.-controlled, adaptive Phase I/II trial in Belgium, Spain, Portugal and others.

Randomised
Yes
Open Label
Yes
Comparator
Placebo (DNL593 Placebo, Sterile lyophilisate in single-dose glass vials). No dose or schedule for placebo specified in provided data.
Adaptive
True, includes dose-escalation design (Part A Dose Escalation) and protocol-specified cohort dose-stopping criteria (e.g. per Protocol Section 4.1.5 cohort stopping criteria: halt dosing if two participants experience severe (Grade 3 or higher) or serious AEs related to infusion-related reactions). Dose Escalation Committee (DEC-B) reviews and can pause dosing/screening/enrolment.
Single Multiple Or Escalation Dose Combined
Yes
Target Sample Size
55
Trial Duration For Participant
540

Eligibility

Recruits 55 Participants with cognitive impairment (frontotemporal dementia) are identified as a vulnerable population. The trial includes study-partner / caregiver informed consent documents and a Study-Partner authorization form indicating that study partners/caregivers are involved in consent/authorization processes where appropriate. Multiple participant-facing ICFs (including Pregnancy-and-Newborn, Pre-Screening Genetic Test, Study-Partner/caregiver ICFs) are provided in several languages..

Pregnancy Exclusion
• Have a positive serum pregnancy test or are currently lactating or breastfeeding
Vulnerable Population
Participants with cognitive impairment (frontotemporal dementia) are identified as a vulnerable population. The trial includes study-partner / caregiver informed consent documents and a Study-Partner authorization form indicating that study partners/caregivers are involved in consent/authorization processes where appropriate. Multiple participant-facing ICFs (including Pregnancy-and-Newborn, Pre-Screening Genetic Test, Study-Partner/caregiver ICFs) are provided in several languages.

Inclusion criteria

  • {"criterion_text":"- Part A: - Women of non-childbearing potential (surgically sterilized or post menopausal) or men, aged ≥18 to ≤ 55 years\n- BMI of ≥ 18 to ≤ 32 kg/m²\n- When engaging in sex with a woman of child bearing potential, both the male participant and his female partner must use highly effective contraception\n- Part B: - Women of non-childbearing potential (surgically sterilized or post menopausal) or men, aged ≥18 to ≤ 80 years. Women who are of childbearing potential but on highly effective, low user dependent contraceptive methods will be allowed. \n- BMI of ≥ 18 to ≤ 32 kg/m²\n- Have a Clinical Dementia Rating® plus National Alzheimer's Coordinating Center frontotemporal lobar degeneration global score ≥ 0.5 \n- Have confirmed granulin (GRN) mutation via genetic testing or historical records available for review by investigator\n- When engaging in sex with a woman of child bearing potential, both the male participant and his female partner must use highly effective contraception\n- Part C: All participants who completed Part B of this trial are eligible for an 18-month OLE if the participant has no unresolved clinically significant TEAEs, where continued dosing may represent a risk to participant safety."}

Exclusion criteria

  • {"criterion_text":"- • Have any history of clinically significant neurologic, psychiatric, endocrine, pulmonary, cardiovascular, gastrointestinal, hepatic, pancreatic, renal, metabolic, hematologic, immunologic, or allergic disease, or other major disorders\n- • Have a history of malignancy, except fully resected basal cell carcinoma or other malignancies at low risk of recurrence \n- • Have a clinically significant history of stroke, cognitive impairment due to causes other than FTD, seizure within 5 years of screening, or head trauma with loss of consciousness within 2 years of screening \n- • Have a positive serum pregnancy test or are currently lactating or breastfeeding"}

Endpoints

Primary endpoints

  • {"endpoint_text":"- Incidence, severity, and seriousness of treatment-emergent adverse events (TEAEs) [Timeframe: Up to 18 months]","definition_or_measurement_approach":"Assessment of incidence, severity and seriousness of TEAEs recorded during study participation; Timeframe stated as Up to 18 months."}
  • {"endpoint_text":"- Change from baseline in safety laboratory values, vital sign measurements, ECG results, and physical/neurological examination findings [Timeframe: Up to 18 months]","definition_or_measurement_approach":"Change from baseline measured in laboratory safety tests, vital signs, ECGs and physical/neurological examinations; Timeframe stated as Up to 18 months."}

Secondary endpoints

  • {"endpoint_text":"- DNL593 serum PK parameters (when feasible): Cmax, tmax, Ctrough, AUClast, AUCt , t 1/2, Accumulation ratio [Timeframe: Up to 18 months]","definition_or_measurement_approach":"Standard PK parameter measurement in serum (Cmax, tmax, Ctrough, AUClast, AUCt, t1/2, accumulation ratio); Timeframe Up to 18 months."}
  • {"endpoint_text":"- Concentration of DNL593 in cerebrospinal fluid (CSF) [Timeframe: Up to 18 months]","definition_or_measurement_approach":"Measurement of DNL593 concentration in CSF samples; Timeframe Up to 18 months."}
  • {"endpoint_text":"- DNL593 CSF:serum concentration ratio [Timeframe: Up to 18 months]","definition_or_measurement_approach":"Ratio of measured DNL593 concentration in CSF relative to serum; Timeframe Up to 18 months."}
  • {"endpoint_text":"- Percentage change from baseline in plasma Nfl at Week 25","definition_or_measurement_approach":"Percent change from baseline in plasma neurofilament light (NfL) measured at Week 25."}

Recruitment

Digital Remote Recruitment
True, recruitment materials indicate use of digital or remote methods including email communications, telephone pre-screening and consent procedures, and vendor tools such as ScoutPass reloadable cards and electronic communications referenced in recruitment documents.
Planned Sample Size
55
Recruitment Window Months
69
Consent Approach
Informed consent obtained from adult participants (all participants aged ≥18). Study includes dedicated participant ICFs (Main ICF Part B/C), Pre-Screening Genetic Test ICF, Pregnancy-and-Newborn ICF, Travel Reimbursement ICF and Study-Partner/caregiver ICFs. For vulnerable participants with FTD, study-partner / caregiver ICFs and a Study-Partner authorization form are provided to handle consent/authorization as appropriate. Participant-facing documents are available in multiple languages (examples in English, Dutch, French, Spanish, Portuguese, Italian, Czech).

Methods

  • Referral letters to clinicians / GPs (documented: Referral-Letter and GP letter documents listed per country).
  • Recruitment arrangements documents/K1 templates per country (e.g., K1_Recruitment-Arrangements for BE, ES, IT, NL, PT, CZ) describing site-based recruitment procedures.
  • Site-based recruitment via participating neurology clinics and hospitals (site lists and contact persons provided).
  • Vendor-supported recruitment materials and incentives (ScoutPass reloadable, ScoutPass materials and Study Brochure referenced in country documents).
  • Telephone contact and pre-ICF telephone consent/data-consent procedures (Pre-ICF Telephone-Data-Consent documents present).
  • Email communication (S495-SC-PFD-Email Communication document referenced).

Geography

Total Number Of Sites
18
Total Number Of Participants
55

Belgium

Earliest CTIS Part Ii Submission Date
29-04-2024
Latest Decision Or Authorization Date
24-02-2026
Processing Time Days
666
Number Of Sites
2
Number Of Participants
5

Sites

Site Name
Antwerp University Hospital
Department Name
Neurology
Principal Investigator Name
Rose Bruffaerts
Principal Investigator Email
rose.bruffaerts@uantwerpen.be
Contact Person Name
Rose Bruffaerts
Contact Person Email
rose.bruffaerts@uantwerpen.be
Site Name
UZ Leuven
Department Name
Neurology
Principal Investigator Name
Rik Vandenberghe
Principal Investigator Email
rik.vandenberghe@uzleuven.be
Contact Person Name
Rik Vandenberghe
Contact Person Email
rik.vandenberghe@uzleuven.be

Spain

Earliest CTIS Part Ii Submission Date
29-04-2024
Latest Decision Or Authorization Date
27-02-2026
Processing Time Days
669
Number Of Sites
3
Number Of Participants
15

Sites

Site Name
University Hospital Virgen Del Rocio S.L.
Department Name
Neurology Department
Principal Investigator Name
Emilio Franco Macías
Principal Investigator Email
efranco17@gmail.com
Contact Person Name
Emilio Franco Macías
Contact Person Email
efranco17@gmail.com
Site Name
Hospital Universitario Donostia
Department Name
Neurology Department
Principal Investigator Name
Fermín Moreno Izco
Principal Investigator Email
fermin.morenoizco@osakidetza.eus
Contact Person Name
Fermín Moreno Izco
Site Name
Hospital Clinic De Barcelona
Department Name
Neurology Department
Principal Investigator Name
Raquel Sánchez del Valle Díaz
Principal Investigator Email
rsanchez@clinic.cat
Contact Person Name
Raquel Sánchez del Valle Díaz
Contact Person Email
rsanchez@clinic.cat

Portugal

Earliest CTIS Part Ii Submission Date
29-04-2024
Latest Decision Or Authorization Date
24-02-2026
Processing Time Days
666
Number Of Sites
3
Number Of Participants
7

Sites

Site Name
Hospital Pedro Hispano
Department Name
Departamento de Neurologia
Principal Investigator Name
Vítor Cruz
Principal Investigator Email
vitor.tedimcruz@ulsm.min-saude.com
Contact Person Name
Vítor Cruz
Site Name
CNS Saude Lda.
Department Name
Unidade de Demências
Principal Investigator Name
Joana Morgado
Principal Investigator Email
joanacmorgado@gmail.com
Contact Person Name
Joana Morgado
Contact Person Email
joanacmorgado@gmail.com
Site Name
CCAB Centro Clinico Academico Braga Associacao
Department Name
Serviço de Neurologia
Principal Investigator Name
Álvaro Machado
Principal Investigator Email
alvmac@gmail.com
Contact Person Name
Álvaro Machado
Contact Person Email
alvmac@gmail.com

Netherlands

Earliest CTIS Part Ii Submission Date
29-04-2024
Latest Decision Or Authorization Date
25-02-2026
Processing Time Days
667
Number Of Sites
1
Number Of Participants
3

Sites

Site Name
Erasmus Universitair Medisch Centrum Rotterdam (Erasmus MC)
Department Name
Neurology
Principal Investigator Name
Harro Seelaar
Principal Investigator Email
h.seelaar@erasmusmc.nl
Contact Person Name
Harro Seelaar
Contact Person Email
h.seelaar@erasmusmc.nl

Czechia

Earliest CTIS Part Ii Submission Date
30-07-2025
Latest Decision Or Authorization Date
19-09-2025
Processing Time Days
51
Number Of Sites
1
Number Of Participants
5

Sites

Site Name
Fakultni Nemocnice V Motole
Department Name
Neurologická klinika
Principal Investigator Name
Jakub Hort
Principal Investigator Email
jakub.hort@gmail.com
Contact Person Name
Jakub Hort
Contact Person Email
jakub.hort@gmail.com

Italy

Earliest CTIS Part Ii Submission Date
29-04-2024
Latest Decision Or Authorization Date
27-02-2026
Processing Time Days
669
Number Of Sites
4
Number Of Participants
12

Sites

Site Name
Istituto Auxologico Italiano IRCCS
Department Name
U.O. Neurologia e Laboratorio di Neuroscienze
Principal Investigator Name
Vincenzo Silani
Principal Investigator Email
vincenzo.silani@unimi.it
Contact Person Name
Vincenzo Silani
Contact Person Email
vincenzo.silani@unimi.it
Site Name
Azienda Socio Sanitaria Territoriale Degli Spedali Civili Di Brescia
Department Name
Unità Operativa di Neurologia
Principal Investigator Name
Salvatore Caratozzolo
Contact Person Name
Salvatore Caratozzolo
Site Name
Pia Fondazione Di Culto E Religione Card G Panico
Department Name
U.O.C. Malattie Neurodegenerative
Principal Investigator Name
Daniele Urso
Principal Investigator Email
d.ursoo@piafondazionepanico.it
Contact Person Name
Daniele Urso
Contact Person Email
d.ursoo@piafondazionepanico.it
Site Name
Careggi University Hospital
Department Name
Dipartimento Neuromuscoloscheletrico e degli organi di senso - (Padiglione 25 CTO-Neuromotorio)
Principal Investigator Name
Valentina Bessi
Principal Investigator Email
valentina.bessi@unifi.it
Contact Person Name
Valentina Bessi
Contact Person Email
valentina.bessi@unifi.it

France

Earliest CTIS Part Ii Submission Date
29-04-2024
Latest Decision Or Authorization Date
02-03-2026
Processing Time Days
673
Number Of Sites
4
Number Of Participants
8

Sites

Site Name
Assistance Publique Hopitaux De Paris
Department Name
Institut de la mémoire et de la maladie d’Alzheimer
Principal Investigator Name
Isabelle LE BER
Principal Investigator Email
isabelle.leber@upmc.fr
Contact Person Name
Isabelle LE BER
Contact Person Email
isabelle.leber@upmc.fr
Site Name
Centre Hospitalier Universitaire De Nantes
Department Name
CentreMémoire Ressources et Recherche
Principal Investigator Name
Claire Boutoleau-Bretonnière
Principal Investigator Email
claire.boutoleaubretonniere@chu-nantes.fr
Contact Person Name
Claire Boutoleau-Bretonnière
Site Name
Centre Hospitalier Universitaire De Toulouse
Department Name
Department of Neurology
Principal Investigator Name
Jérémie Pariente
Principal Investigator Email
jeremie.pariente@inserm.fr
Contact Person Name
Jérémie Pariente
Contact Person Email
jeremie.pariente@inserm.fr
Site Name
Centre Hospitalier Universitaire Rouen
Department Name
Department of Neurology
Principal Investigator Name
David Wallon
Principal Investigator Email
david.wallon@chu-rouen.fr
Contact Person Name
David Wallon
Contact Person Email
david.wallon@chu-rouen.fr

Sponsor

Primary sponsor

Full Name
Denali Therapeutics Inc.
Organisation Type
Pharmaceutical company
Country Of Registered Address
United States

Contract research organisations

Name
PPD Development L.P.
Name
Icon Clinical Research Limited
Name
IQVIA Limited
Name
Fisher Clinical Services GmbH

Third parties

  • {"country":"United States","full_name":"PPD Development L.P.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"Australia","full_name":"Cogstate Limited","duties_or_roles":"Patient Material (Raters)","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Eresearchtechnology Inc.","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Preventiongenetics LLC","duties_or_roles":"Genetic Screening","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"Kcas LLC","duties_or_roles":"Exploratory Endpoint Analysis","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Thermo Fisher Scientific Inc.","duties_or_roles":"Clinical supply","organisation_type":"Pharmaceutical company"}
  • {"country":"Germany","full_name":"Fisher Clinical Services GmbH","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"Primevigilance Limited","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Aperio Clinical Outcomes LLC","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Scout Clinical","duties_or_roles":"Patient Reimbursement","organisation_type":"Hospital/Clinic/Other health care facility"}
  • {"country":"Australia","full_name":"Redenlab Pty Limited","duties_or_roles":"Language assessments","organisation_type":"Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"IQVIA Limited","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"Ireland","full_name":"Icon Clinical Research Limited","duties_or_roles":"","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Bioagilytix Labs LLC","duties_or_roles":"Primary / Surrogate endopoints test; Bioanalysis","organisation_type":"Pharmaceutical company"}

Investigational products

Investigational Product Name
DNL593
Active Substance
DNL593
Modality
Peptide/protein/enzyme
Routes Of Administration
Intravenous
Route
Intravenous
Authorisation Status
Investigational; MIA DE_BW_01_MIA_2021_0139 (as provided in product information)
Investigational Product Name
DNL593 Placebo, Sterile lyophilisate in single-dose glass vials
Modality
Other
Authorisation Status
Placebo (not applicable)

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