Actively Recruiting

Phase 2
Age: 18Years +
All Genders
NCT05650229

Efficacy of KL1333 in Adult Patients With Primary Mitochondrial Disease

Led by Abliva AB · Updated on 2026-04-15

180

Participants Needed

35

Research Sites

254 weeks

Total Duration

On this page

AI-Summary

What this Trial Is About

The primary objective of the FALCON study is to evaluate the efficacy of KL1333 on selected disease manifestations of primary mitochondrial disease (PMD) following 48 weeks of treatment. This objective involves evaluating the efficacy of KL1333 versus placebo on fatigue symptoms and impacts on daily living as well as on functional lower extremity strength and endurance. Additionally, the study evaluates the safety and tolerability of KL1333.

CONDITIONS

Official Title

Efficacy of KL1333 in Adult Patients With Primary Mitochondrial Disease

Who Can Participate

Age: 18Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Age 18 years or older
  • Confirmed diagnosis of primary mitochondrial disease caused by a known pathogenic gene mutation or mitochondrial genome deletion with multisystemic symptoms including MELAS-MIDD, KSS-CPEO, or MERRF
  • History of mitochondrial fatigue for at least 3 months prior to screening
  • At least moderate fatigue level with PROMIS Fatigue PMD Short form raw score 63 27 at screening and baseline
  • Presence of mitochondrial myopathy defined by minimal proximal muscle weakness or exercise tolerance symptoms
  • Ability to perform at least 2 repetitions and not exceed 17 (male) or 16 (female) repetitions in a 30-second sit-to-stand test at screening
  • Clinically stable apart from mitochondrial disease symptoms as determined by medical evaluations
  • Willingness and ability to attend study appointments within time windows
  • Willingness and ability to complete electronic patient-reported outcomes
  • Willingness to maintain stable diet during screening and study
  • Stable dose regimen for 3 months prior to randomisation of mitochondrial disease-focused vitamins or supplements like CoQ10, vitamin B3, and L-arginine and intention to maintain during study
  • Willingness to suspend idebenone treatment during study
  • Female participants not pregnant and either not of childbearing potential or using highly effective contraception from consent through 36 days after last dose
  • Male participants with female partners of childbearing potential agree to use condoms plus highly effective contraception during treatment and for 96 days after last dose
  • Female participants agree not to breastfeed during study and for 36 days after last dose
  • Female participants agree not to donate ova and male participants agree not to donate sperm during study and respective post-treatment periods
Not Eligible

You will not qualify if you...

  • Primary mitochondrial disease with mainly neurodegenerative forms such as Leigh syndrome, Leber hereditary optic neuropathy, and NARP
  • Nuclear DNA mutations causing PMD or uncertain mitochondrial DNA variants
  • Fatigue or muscle weakness from causes other than mitochondrial disease
  • Significant cardiovascular disease or clinically significant abnormal ECG including QTcF above threshold
  • Recent unstable disease or uncontrolled neurological symptoms including recent stroke-like episodes, frequent seizures, status epilepticus hospitalization, or frequent migraines
  • History of inflammatory bowel disease, gastric erosions, peptic ulcers, gastrointestinal bleeding, or symptomatic gastroesophageal reflux disease in last 6 months
  • Laboratory abnormalities that pose safety risks including low kidney function or elevated liver enzymes outside allowed limits
  • Severe ataxia, neuropathy, balance problems, or other conditions interfering with sit-to-stand test
  • Untreated or undertreated sleep apnea
  • Use of idebenone within 14 days prior to first dose
  • History of unstable or severe pulmonary, immunological, oncological, hepatic, or renal disease or other major illness that could interfere with study
  • Unlikely to comply with protocol due to cognitive or other reasons
  • Immediate family member participating in study
  • Positive pregnancy test at screening or baseline
  • Use of investigational drugs within 30 days or 5 half-lives prior to screening or planned use during study
  • Hypersensitivity to study drug or placebo ingredients

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Trial Site Locations

Total: 35 locations

1

University of California, Irvine - ALS & Neuromuscular Center

Orange, California, United States, 92868

Actively Recruiting

2

The Regents of the University of California - San Diego

San Diego, California, United States, 292093

Actively Recruiting

3

Children's Hospital Colorado - Center for Cancer and Blood Disorders (CCBD) - Anschutz Medical Campus Location

Aurora, Colorado, United States, 80045

Actively Recruiting

4

Rare Disease Research, LLC

Atlanta, Georgia, United States, 303129

Actively Recruiting

5

Mayo Clinic

Rochester, Minnesota, United States, 55905

Actively Recruiting

6

Washington University School of Medicine - Center for Advanced Medicine (CAM) - Neuroscience Center

St Louis, Missouri, United States, 63110

Not Yet Recruiting

7

Columbia University Irving Medical Center

New York, New York, United States, 100032

Not Yet Recruiting

8

Akron Children's Hospital

Akron, Ohio, United States, 44307

Actively Recruiting

9

UPMC Children's Hospital of Pittsburgh

Pittsburgh, Pennsylvania, United States, 15224

Not Yet Recruiting

10

Baylor College of Medicine (BCM)

Houston, Texas, United States, 77030

Actively Recruiting

11

The University of Texas Health Science Center at Houston

Houston, Texas, United States, 77030

Actively Recruiting

12

Hopital Universitaire de Bruxelles (H.U.B)/ Academisch Ziekenhuis Brussel

Brussels, Belgium

Actively Recruiting

13

Universitair Ziekenhuis Gent

Ghent, Belgium

Actively Recruiting

14

Universitair Ziekenhuis Leuven Gasthuisberg Campus

Leuven, Belgium

Actively Recruiting

15

Copenhagen Neuromuscular Center, Rigshospitalet

Copenhagen, Denmark

Actively Recruiting

16

Centre Hospitalier Universitaire (CHU) de Bordeaux - Groupe Hospitalier Pellegrin

Bordeaux, France

Actively Recruiting

17

Hopital Roger Salengro, CHRU de Lille

Lille, France

Actively Recruiting

18

Centre Hospitalier Universitaire de Nice, Hopital Pasteur 2

Nice, France

Actively Recruiting

19

CHU de NICE - Hôpital Archet 2

Nice, France

Actively Recruiting

20

Groupe Hospitalier Pitie-Salpetriere

Paris, France

Actively Recruiting

21

Charite - Universitaetsmedizin Berlin

Berlin, Germany, 10117

Actively Recruiting

22

Universitaetsklinikum Halle

Halle, Germany, 6120

Actively Recruiting

23

IRCCS Institute of Neurological Sciences of Bologna- Universita di Bologna

Bologna, Italy, 40139

Actively Recruiting

24

Azienda Ospedaliera Universitaria Gaetano Martino Messina

Messina, Italy, 98125

Actively Recruiting

25

Fondazione IRCCS Istituto Neurologico Carlo Besta

Milan, Italy, 20133

Actively Recruiting

26

Azienda Ospedaliero Universitaria Pisana

Pisa, Italy, 56126

Actively Recruiting

27

Fondazione Policlinico Universitario Agostino Gemelli IRCCS - Universita Cattolica del Sacro Cuore

Roma, Italy, 00168

Actively Recruiting

28

Radboud University Medical Center

Nijmegen, Netherlands, 6525

Actively Recruiting

29

CIBERER- IDIBAPS, Faculty of Medicine, University of Barcelona

Barcelona, Spain

Actively Recruiting

30

Hospital de la Santa Creu i Sant Pau

Barcelona, Spain

Completed

31

Hospital General Universitario de Catalunya

Barcelona, Spain

Actively Recruiting

32

Hospital Universitario 12 de Octubre

Madrid, Spain

Actively Recruiting

33

Department of Clinical Neurosciences, Addenbrooke's Hospital

Cambridge, United Kingdom

Actively Recruiting

34

University College London Hospitals Nhs Foundation Trust

London, United Kingdom

Actively Recruiting

35

Royal Victoria Infirmary - The Newcastle Upon Tyne Hospitals NHS Foundation Trust

Newcastle, United Kingdom

Actively Recruiting

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Research Team

C

Communication Manager

CONTACT

How is the study designed?

Study Type

INTERVENTIONAL

Masking

QUADRUPLE

Allocation

RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

2

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Efficacy of KL1333 in Adult Patients With Primary Mitochondrial Disease | DecenTrialz