Evidence map›Paper›PMID 40474241›Full record

ReviewOrphanet journal of rare diseases2025

Gene therapy of rare diseases as a milestone in medicine - overview of the field and report on initial experiences in Slovenia.

Urh Grošelj, Marko Kavčič, Ana Drole Torkar, Jan Kafol, Duško Lainšček, Roman Jerala, Matjaž Sever, Samo Zver, Gregor Serša, Maja Čemažar and 18 more

Registry-linked trialAbstract readReview
In one paragraph

Review in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07270549 (GAIN-CTNNB1), which is not on this map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT07270549 phase1 / phase2recruitingnot on this map

GAIN-CTNNB1: A Phase I/II Open-Label Trial To Evaluate the Safety, Tolerability, and Preliminary Efficacy of Intracerebroventricular Administration of an AAV9 Based Gene Replacement Therapy in Paediatric Patients With CTNNB1 Neurodevelopmental Syndrome

TypeinterventionalSponsorCTNNB1 FoundationRan2025 to 2032Enrolled12ConditionsCTNNB1 Neurodevelopmental SyndromeArmsUrbagen gene addition therapy, Sirolimus, Methylprednisolone (Corticosteroid)
3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Review
  2. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

28 authors.

Urh Grošelj *Department of Endocrinology, Diabetes, and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia. urh.groselj@kclj.si.ORCID http://orcid.org/0000-0002-5246-9869
Marko Kavčič *Department of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-4949-1344
Ana Drole TorkarDepartment of Endocrinology, Diabetes, and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia.ORCID http://orcid.org/0000-0002-0506-2919
Jan KafolFaculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0009-0003-4159-1783
Duško LainščekDepartment of Synthetic Biology and Immunology, National Institute of Chemistry, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0001-9613-5441
Roman JeralaDepartment of Synthetic Biology and Immunology, National Institute of Chemistry, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-6337-5251
Matjaž SeverFaculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Samo ZverFaculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0003-2932-7923
Gregor SeršaInstitute of Oncology Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-7641-5670
Maja ČemažarInstitute of Oncology Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-1418-1928
Primož StrojanInstitute of Oncology Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-0445-112X
Aleš GrošeljDepartment of Otorhinolaryngology and Cervicofacial Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Mojca Žerjav TanšekDepartment of Endocrinology, Diabetes, and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia.ORCID http://orcid.org/0000-0002-3304-5120
Špela MiroševičDepartment of Family Medicine, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0001-5004-0607
Simona IvančanDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Tomaž PrelogDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
David GosarDepartment of Paediatric Neurology, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia.
Jasna Oražem MrakDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Matej MlinaričDepartment of Endocrinology, Diabetes, and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia.ORCID http://orcid.org/0000-0003-1143-2880
Sara BertokDepartment of Endocrinology, Diabetes, and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia.
Jernej KovačDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0003-3080-4838
Jana KodričChild Psychiatry Unit, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Saba BattelinoFaculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0003-1478-0573
Marko PokornDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-2341-2791
Alojz IhanInstitute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0003-2904-1212
Janez JazbecDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.ORCID http://orcid.org/0000-0002-7426-1591
Tadej BattelinoDepartment of Endocrinology, Diabetes, and Metabolic Diseases, University Children's Hospital, University Medical Centre Ljubljana, Bohoriceva 20, Ljubljana, 1000, Slovenia.ORCID http://orcid.org/0000-0002-0273-4732
Damjan OsredkarDepartment of Paediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia. damjan.osredkar@kclj.si.ORCID http://orcid.org/0000-0002-2188-420X

Funding

Javna Agencija za Raziskovalno Dejavnost RS J3-4526Javna Agencija za Raziskovalno Dejavnost RS J4-4563Javna Agencija za Raziskovalno Dejavnost RS J7-4537Javna Agencija za Raziskovalno Dejavnost RS J7-4640Javna Agencija za Raziskovalno Dejavnost RS L3-50111Javna Agencija za Raziskovalno Dejavnost RS P3-0003Javna Agencija za Raziskovalno Dejavnost RS P3-0343Ministrstvo za visoko šolstvo, znanost in tehnologijo 3330-18-1115Research Executive Agency 101059842
6 · The paper itself

Abstract

Gene therapy has transitioned from a long-awaited promise to a clinical reality, offering transformative treatments for rare congenital diseases and certain cancers, which have a significant impact on patients' lives. Current approaches focus on gene replacement therapy, either in vivo or ex vivo, mostly utilizing viral vectors to deliver therapeutic genes into target cells. However, refining these techniques is essential to overcome challenges and complications associated with gene therapy to ensure long-term safety and efficacy. Slovenia has witnessed significant advancements in this field since 2018, marked by successful gene therapy trials and treatments for various rare diseases. Significant strides have been made in the field of gene therapy in Slovenia, treating patients with spinal muscular atrophy and rare metabolic disorders, including the pioneering work on CTNNB1 syndrome. Additionally, immune gene therapy, exemplified by IL-12 adjuvant therapy for cancer, has been a focus of research in Slovenia. Through patient-centred initiatives and international collaborations, researchers in Slovenia are advancing preclinical research and clinical trials, paving the way for accessible gene therapies. Establishing clinical infrastructure and genomic diagnostics for rare diseases is crucial for gene therapy implementation. Efforts in this regard in Slovenia, including the establishment of a Centre for Rare Diseases, Centre for the Technologies of Gene and Cell Therapy, and rapid genomic diagnostics, demonstrate a commitment to comprehensive patient care. Despite the promises of gene therapy, challenges remain, including cost, distribution, efficacy, and long-term safety. Collaborative efforts are essential to address these challenges and ensure equitable access to innovative therapies for patients with rare diseases.

Indexed as

Genetic TherapyRare DiseasesHumansSloveniaCancerCAR-T cellsCRISPR/Cas9CTGCTCTNNB1 syndromeGene therapyImmune gene therapyInterleukin 12Rare genetic diseasesSlovenia

Identifiers

PMID40474241
PMCPMC12142986

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.