Clinical trial • Phase II • Neurology|Rare Disease|Musculoskeletal

APITEGROMAB for Spinal muscular atrophy

Phase II trial of APITEGROMAB for Spinal muscular atrophy. None/Not specified-controlled. 22 participants.

Overview

Trial Therapeutic Area
Neurology|Rare Disease|Musculoskeletal
Trial Disease
Spinal muscular atrophy
Trial Stage
Phase II
Drug Modality
Monoclonal antibody
Paediatric Trial
Yes
Orphan Drug
Yes

Key dates

Initial CTIS Submission Date
02-07-2025
First CTIS Authorization Date
21-10-2025

Trial design

None/Not specified-controlled Phase II trial in Belgium, France, Italy and others.

Comparator
None/Not specified
Target Sample Size
22
Trial Duration For Participant
336

Eligibility

Recruits 22 paediatric patients.

Vulnerable Population
Participants are infants/toddlers (<2 years old); vulnerable population selected. Informed consent is provided by parent/guardian (Parent-guardian ICF and Subject Information Sheets are available in multiple languages). Assent is not applicable due to participant age.

Inclusion criteria

  • {"criterion_text":"- Is <2 years old at the time of the informed consent\n- Had a gestational age of ≥35 weeks and gestational body weight ≥2.0 kg at birth\n- Has confirmed diagnosis of 5q autosomal recessive SMA\n- Has confirmed presence of SMN2 gene copy(ies)\n- Must have been treated with an approved SMN1-targeted therapy (ie, onasemnogene abeparvovec-xioi) or are continuing to be treated with an approved SMN2-targeted therapy (ie, nusinersen or risdiplam)\n- Body weight for age is no less than 1st percentile based on the WHO Child Growth Standards at the Screening Visit\n- Has delayed motor milestones for age attributed to SMA at the discretion of the Investigator or a CHOP-INTEND score <55."}

Exclusion criteria

  • {"criterion_text":"- Nutritional status that is not anticipated to be stable throughout the study or medical necessity for a gastric feeding tube, where most feeds are administered by this route, as assessed by the Investigator and/or an Oral and Swallowing Abilities Tool score of 0.\n- Major orthopedic issues such as severe scoliosis or severe contractures or interventional procedure, including spine or hip surgery, which is considered to have the potential to substantially limit the ability of the subject to be evaluated on any motor function outcome measures, within 6 months before Screening or anticipated during the study.\n- Any other physical limitations (eg, the subject requires cast for contractures) that would prevent the subject from undergoing motor function outcome measures throughout the study."}

Endpoints

Primary endpoints

  • {"endpoint_text":"- Population PK model-based parameters such as CL, Vd, Vc and Vp","definition_or_measurement_approach":"Population pharmacokinetic (PK) model-based estimation of parameters (e.g., CL, Vd, Vc, Vp) from collected PK data."}
  • {"endpoint_text":"- Apitegromab and total latent myostatin concentrations in serum","definition_or_measurement_approach":"Measurement of apitegromab and total latent myostatin concentrations in serum (serum concentration assays)."}
  • {"endpoint_text":"- Change from baseline in the raw score of the BSID-4 GMS at 48 weeks","definition_or_measurement_approach":"Assessment of change from baseline in Bayley Scales of Infant and Toddler Development, 4th Edition (BSID-4) Gross Motor Scale (GMS) raw score at 48 weeks."}

Secondary endpoints

  • {"endpoint_text":"- Relationship of apitegromab concentrations and total latent myostatin concentration in serum","definition_or_measurement_approach":"Analysis of the relationship/correlation between apitegromab serum concentrations and total latent myostatin serum concentrations."}
  • {"endpoint_text":"- Population PK/PD model-based parameters such as Imax, IC50, and Kout","definition_or_measurement_approach":"Population PK/PD modeling to estimate parameters (e.g., Imax, IC50, Kout) from PK and PD data."}
  • {"endpoint_text":"- Incidence of TEAEs and SAEs by severity","definition_or_measurement_approach":"Collection and tabulation of treatment-emergent adverse events (TEAEs) and serious adverse events (SAEs) categorized by severity."}

Recruitment

Registry Or Advocacy Recruitment
True, Advocacy PAG (patient advocacy group) clinical trial listing materials are used
Digital Remote Recruitment
True, includes website pages, social media posts, and e-newsletter content in multiple languages for outreach
Planned Sample Size
22
Recruitment Window Months
40
Consent Approach
Informed consent obtained from parent/guardian using Parent-guardian ICF and Subject Information Sheets (L1 SIS and ICF Parent-Guardian documents). ICF/SIS materials available in multiple languages (English, French, Dutch, Spanish, Italian as per document titles). Assent is not applicable due to participant age (<2 years).

Methods

  • Website text – country-specific website recruitment pages (documents: Website_BE_DUT, Website_BE_FRE, Website_FRE_Redacted, Website_IT_Redacted, Website_SPA_Redacted).
  • Social media posts – country/language specific social media recruitment posts (documents: Social media posts_BE_DUT, _BE_FRE, _IT, _SPA).
  • Posters with flyer – printed poster/flyer materials for clinics (documents: Poster with flyer_BE_FRE, _BE_DUT, _IT, _SPA).
  • Dr to parent/guardian letter – clinician-to-parent invitation letters (documents: Dr to parent guardian letter_BE_FRE, _BE_DUT, _IT, _SPA).
  • Parent/guardian brochure – information brochures for parents/guardians (documents: Parent guardian brochure_BE_DUT_Redacted, _BE_FRE_Redacted, Parent-Guardian brochure_Redacted, parent_guardian_brochure_IT_Redacted, Parent-Guardian Brochure_SPA_Redacted).
  • Advocacy/PAG clinical trial listing – materials for patient advocacy groups (documents: Advocacy PAG clinical trial listing_BE_DUT_Redacted, _BE_FRE_Redacted, Clinical Trial Listing_SPA_Redacted, advocacy materials_IT).
  • E-newsletter / eNewsletter content – electronic newsletter content for advocacy/targeted audiences (documents: PAG enewsletter_BE_DUT, _BE_FRE, eNewsletter Content_SPA, eNewsletter content_IT).
  • GP/clinician letters and clinic-based recruitment – GP letter and clinic referral materials (documents: GP letter_IT, Dr to Parent-Guardian letters).

Geography

Total Number Of Sites
11
Total Number Of Participants
30

Belgium

Earliest CTIS Part Ii Submission Date
10-10-2025
Latest Decision Or Authorization Date
21-10-2025
Processing Time Days
11
Number Of Sites
3
Number Of Participants
6

Sites

Site Name
UZ Leuven
Department Name
Neuromuscular Diseases
Principal Investigator Name
Liesbeth De Waele
Principal Investigator Email
Liesbeth.dewaele@uzleuven.be
Contact Person Name
Liesbeth De Waele
Contact Person Email
Liesbeth.dewaele@uzleuven.be
Site Name
Universitair Ziekenhuis Gent
Department Name
Neuromusculair Referentiecentrum
Principal Investigator Name
Nicolas Deconinck
Principal Investigator Email
Nicolas.deconinck@uzgent.be
Contact Person Name
Nicolas Deconinck
Contact Person Email
Nicolas.deconinck@uzgent.be
Site Name
Centre Hospitalier Regional De La Citadelle
Department Name
Neuro paediatry
Principal Investigator Name
Aurore Daron
Principal Investigator Email
Aurore.daron@chrcitadelle.be
Contact Person Name
Aurore Daron
Contact Person Email
Aurore.daron@chrcitadelle.be

France

Earliest CTIS Part Ii Submission Date
25-08-2025
Latest Decision Or Authorization Date
24-10-2025
Processing Time Days
60
Number Of Sites
2
Number Of Participants
8

Sites

Site Name
Association Institut De Myologie
Department Name
I-Motion, Hopital Armand Trousseau
Principal Investigator Name
Andreea SEFERIAN
Principal Investigator Email
a.seferian@institut-myologie.org
Contact Person Name
Andreea SEFERIAN
Site Name
Centre Hospitalier Universitaire De Lille
Department Name
Hopital Jeanne de Flandre - Centre d investigation clinique pediatrique
Principal Investigator Name
Jean-Baptiste DAVION
Principal Investigator Email
Jeanbaptiste.DAVION@chu-lille.fr
Contact Person Name
Jean-Baptiste DAVION

Italy

Earliest CTIS Part Ii Submission Date
15-07-2025
Latest Decision Or Authorization Date
23-10-2025
Processing Time Days
100
Number Of Sites
3
Number Of Participants
9

Sites

Site Name
Centro Clinico Nemo
Department Name
Centro Clinico Nemo
Principal Investigator Name
Valeria Ada Sansone
Principal Investigator Email
valeria.sansone@centrocliniconemo.it
Contact Person Name
Valeria Ada Sansone
Site Name
Fondazione Policlinico Universitario Agostino Gemelli IRCCS
Department Name
Unita Operativa Complessa di Neuropsichiatria infantile
Principal Investigator Name
Eugenio Maria Mercuri
Principal Investigator Email
eugeniomaria.mercuri@policlinicogemelli.it
Contact Person Name
Eugenio Maria Mercuri
Site Name
IRCCS Foundation Istituto Neurologico Carlo Besta
Department Name
Developmental Neurology Unit
Principal Investigator Name
Riccardo Masson
Principal Investigator Email
riccardo.masson@istituto-besta.it
Contact Person Name
Riccardo Masson

Spain

Earliest CTIS Part Ii Submission Date
01-09-2025
Latest Decision Or Authorization Date
23-10-2025
Processing Time Days
52
Number Of Sites
2
Number Of Participants
6

Sites

Site Name
Hospital Universitario Y Politecnico La Fe
Department Name
Neuromuscular Disease Unit
Principal Investigator Name
Inmaculada Pitarch Castellano
Principal Investigator Email
inmapitarch@hotmail.com
Contact Person Name
Inmaculada Pitarch Castellano
Contact Person Email
inmapitarch@hotmail.com
Site Name
Hospital Sant Joan De Deu Barcelona
Department Name
Neuromuscular Unit
Principal Investigator Name
Andrés Nascimento Osorio
Principal Investigator Email
andres.nascimento@sjd.es
Contact Person Name
Andrés Nascimento Osorio
Contact Person Email
andres.nascimento@sjd.es

Netherlands

Earliest CTIS Part Ii Submission Date
26-09-2025
Latest Decision Or Authorization Date
21-10-2025
Processing Time Days
25
Number Of Sites
1
Number Of Participants
1

Sites

Site Name
Universitair Medisch Centrum Utrecht
Department Name
Department of Neurology
Principal Investigator Name
Ludo Van der Pol
Principal Investigator Email
W.L.vanderPol@umcutrecht.nl
Contact Person Name
Ludo Van der Pol
Contact Person Email
W.L.vanderPol@umcutrecht.nl

Sponsor

Primary sponsor

Full Name
Scholar Rock Inc.
Organisation Type
Pharmaceutical company
Country Of Registered Address
United States

Contract research organisations

Name
Syneos Health Inc.
Responsibilities
Multiple duties (codes present); includes Patient Recruitment, IDMC Oversight
Name
PPD Development LP
Responsibilities
code 8
Name
Charles River Laboratories Inc.
Responsibilities
PK/PD/ADA
Name
Catalent Cts (Kansas City) LLC
Responsibilities
code 14

Third parties

  • {"country":"United States","full_name":"Gray Consulting Inc.","duties_or_roles":"Patient Travel Support","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Eresearchtechnology Inc.","duties_or_roles":"Cardiac Safety","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Syneos Health Inc.","duties_or_roles":"Multiple duties (codes present); includes Patient Recruitment, IDMC Oversight","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"PPD Development LP","duties_or_roles":"code 8","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Medidata Solutions Inc.","duties_or_roles":"code 7","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"Netherlands","full_name":"LabConnect Europe B.V.","duties_or_roles":"Kit Production and Long-Term Sample Storage","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"United States","full_name":"Caerus Marketing Group LLC","duties_or_roles":"Patient Recruitment","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"United Kingdom","full_name":"Chillibean Limited","duties_or_roles":"videotaping","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Charles River Laboratories Inc.","duties_or_roles":"PK/PD/ADA","organisation_type":"Pharmaceutical company"}
  • {"country":"Germany","full_name":"Labor Dr. Wisplinghoff GbR","duties_or_roles":"Short-Term Storage; code 4","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"Ireland","full_name":"Millmount Healthcare Limited","duties_or_roles":"IP shipment and depot","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Catalent Cts (Kansas City) LLC","duties_or_roles":"code 14","organisation_type":"Pharmaceutical company"}
  • {"country":"United States","full_name":"Suvoda LLC","duties_or_roles":"code 3","organisation_type":"Non-Pharmaceutical company"}
  • {"country":"Germany","full_name":"LabConnect GmbH","duties_or_roles":"Short-Term Storage; code 9","organisation_type":"Laboratory/Research/Testing facility"}
  • {"country":"Germany","full_name":"PCI Pharma Services Germany GmbH","duties_or_roles":"IP shipment and depot","organisation_type":"Pharmaceutical company"}

Investigational products

Investigational Product Name
APITEGROMAB
Active Substance
APITEGROMAB
Modality
Monoclonal antibody
Routes Of Administration
INTRAVENOUS USE
Route
INTRAVENOUS USE
Orphan Designation
Yes
Combination Treatment
Yes

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