The new Article of HDBuzz “Scientists identify precisely how pridopidine works in models of Huntington’s disease” by Dr Rachel Harding brings exciting news about PROOF-HD potential study.



Prilenia Neurotherapeutics & Huntington Study Group



PROOF-HD is a Phase 3 clinical study that evaluates the efficacy and safety of pridopidine in patients with early stage of Huntington Disease. Pridopidine is a small molecule developed by Prilenia for the treatment of neurodegenerative disorders such as HD.

According to Prilenia, the Pridopidine is an oral drug administered in a small easy-to-swallow capsule twice a day, which enters the brain and spinal cord, where it activates a protein called the sigma-1 receptor (S1R). Activation of the S1R by pridopidine enhances the clearance of toxic proteins, enhances energy production, and reduces cellular stress and inflammation. These mechanisms are crucial for a neuron’s function and survival.

Webinar: All you need to know about PROOF HD

Dr. Michael Hayden presents Phase 3 PROOF-HD

Ages Eligible
for Study:

25 Years and older

(Adult, Older Adult)

Sexes Eligible
for Study:


Accepts Healthy Volunteers:


  1. Diagnosis of HD based on clinical features and the presence of ≥36 CAG repeats in the huntingtin gene
  2. Diagnostic confidence level (DCL) of 4
  3. Adult-onset HD with onset of signs and symptoms ≥18 years of age
  4. Stage 1 or Stage 2 HD, defined as a UHDRS-TFC score of ≥7, at screening
  1. Use of pridopidine within 12 months before the baseline visit.
  2. Gene therapy at any time
  3. Any serious medical condition or clinically significant laboratory, or vital sign abnormality that precludes the patient’s safe participation in and completion of the study e.g. significant heart disease within 12 weeks before baseline or history of certain cardiac arrhythmias
  4. History of epilepsy or seizures within the last 5 years
  5. Pregnant or breastfeeding, or intention to become pregnant during the study




TRIAL SITE: Medizinische Universität Innsbruck

Adress: Innsbruck

Contact: Dora Valent, Study Site Coordinator
Phone: 0512 504 83237

Czech Republic

TRIAL SITE: Centrum extrapyramidových onemocnění

Adress: Prague

Contact: Jiri Klempir, MD, Study Site Investigator
Tel:   +420  732 273 271


TRIAL SITE: Centre Hospitalier Universitaire (CHU) Henri Mondor

Adress: Paris

Contact: Tiffany Monnier, Study Site Coordinator

TRIAL SITE:Hôpitaux Universitaires de Marseille Timone

Adress: Marseille

Contact: Email: Laura

TRIAL SITE: Centre Hospitalier Universitaire (CHU) of Lille – Hôpital Roger Salengro

Adress: Lille

Contact: Eric Decorte, Study Site Coordinator


TRIAL SITE: Euregional Huntington Center Aachen (EHZA) 

Adress: Aachen

Contact: Maha Sagar, Study Site Coordinator, Email: Tel: +49 241 80 80697

TRIAL SITE: George Huntington Institut

Adress: Münster

Contact: Tel: +49 251 7887880

TRIAL SITE: Huntington Center North Rhine-Westphalia,
Department of Neurology,
Ruhr-University Bochum,
St. Josef-Hospital Bochum

Adress: Bochum

Contact: Email:
Tel.: +49-234-509-2703

TRIAL SITE: Kbo-Isar-Amper-Klinikum Taufkirchen (Vils)

Adress: Taufkirchen (Vils)

Contact: Michael Bachmaier, Study Site Coordinator
Tel: 08084-934-417

TRIAL SITE: University of Lübeck

Adress: Lübeck

Contact: Saruhi Surnaschjan, Study Site Coordinator 
Phone:+49 451 50043497

TRIAL SITE: Ulm University

Adress: Ulm

Sonja Trautmann
Tel:  +49 731 50063083
Ariane Schneider
Tel: +49 731 50063126 


TRIAL SITE: IRCCS Istituto delle Scienze Neurologiche di Bologna

Adress: Bologna

Contact: Tel: +393387367104

TRIAL SITE: Fondazione IRCCS Istituto Neurologico Carlo Besta

Adress: Milano

Contact: Tel: +39 02 23942519

TRIAL SITE: CSS-Mendel Institute at IRCCS Casa Sollievo della Sofferenza Research Hospital

Adress: Roma

Contact: Tel: +39 06 44700887

TRIAL SITE:Universita’ di Bari

Adress: Bari

TRIAL SITE:Università di Napoli Federico II

Adress: Naples

Contact: Email:
Tel: +39 081 5455213

The Netherlands

TRIAL SITE: Maastricht University

Adress: Maastricht 

Contact: Dr. Mayke Oosterloo, Study Site Investigator
Tel: 0031-(0)43-387 76 76, Email:

TRIAL SITE:Leiden University Medical Center

Adress: Leiden

Contact: Tel: 0031715265442 


TRIAL SITE: Szpital Specjalistyczny Swietego Wojciecha

Adress: Gdansk

Contact: Agnieszka Konkel, Study Site Coordinator
Tel: +48 609 952 555

TRIAL SITE: Krakowska Akademia Neurologii

Adress: Krakow

Contact: Email: 
Tel: +48 12 426 92 80

TRIAL SITE:Instytut Psychiatrii i Neurologii

Adress: Warsaw

Contact: Grzegorz Witkowski, Study Site Investigator
Phone: +48694904208


TRIAL SITE:Hospital de la Santa Creu i Sant Pau

Adress: Barcelona

Contact: Dr. Jaime Kulisevsky
Tel: +34 649 14 23 60

TRIAL SITE: Burgos Foundation for Health Research

Adress: Burgos

Contact: Tel:  +34 947 28 18 00 ext 35380

TRIAL SITE: Fundacion Hospital Universitario La Fé

Adress: Valencia

Contact: Francisco Castera, Study Site Coordinator

Carmen PEIRO, Study Site Investigator. Email:
Tel: Phone: +34 682893185

TRIAL SITE:Hospital Ramón y Cajal

Adress: Madrid

Contact: Email:
Tel: +34  638 158 849

United Kingdom

TRIAL SITE: University of Aberdeen, Institute of Medical Sciences

Adress: Aberdeen (Scotland)

Contact: Stella Sihlabela, Study Site Coordinator
Tel: +44 1224 552120

Resifina Seyara, Study Site Coordinator
Tel: +44 1224 552120

TRIAL SITE: Cardiff University

Adress: Cardiff (Wales)

Contact:  Alison Johnson, Study Site Coordinator

TRIAL SITE: Barberry National Centre for Mental Health

Adress: Birmingham (England)

TRIAL SITE: Newcastle upon Tyne Hospitals NHS Foundation

Adress:Newcastle (England)

new FB feed (19)


CAG repeat

The stretch of DNA at the beginning of the HD gene, which contains the sequence CAG repeated many times, and is abnormally long in people who will develop HD. (from

new FB feed (19)


Inclusion Criteria

A type of eligibility criteria. These are the reasons that a person is allowed to participate in a clinical study.

new FB feed (19)



Pridopidine is an oral drug currently in development for the treatment of Huntington’s disease and ALS. It is administered in a small easy-to-swallow capsule twice a day.

Multiple clinical studies have been conducted providing important understanding about pridopidine’s safety, mechanism of action, and efficacy. (From

new FB feed (19)



The stage of a clinical trial studying a drug or biological product, based on definitions developed by the U.S. Food and Drug Administration (FDA). The phase is based on the study’s objective, the number of participants, and other characteristics. There are five phases: Early Phase 1 (formerly listed as Phase 0), Phase 1, Phase 2, Phase 3, and Phase 4. Not Applicable is used to describe trials without FDA-defined phases, including trials of devices or behavioral interventions.

new FB feed (19)


Exclusion Criteria

A type of eligibility criteria. These are reasons that a person is not allowed to participate in a clinical study.