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NCT06582537

LAMA2 Genetic Correction

Completed Last updated 3 September 2024
What this trial tests

trial testing Participants sample collection in MDC1A in 7 participants. Completed in 18 July 2024.

Timeline
1 July 2020
Primary endpoint
18 July 2024
18 July 2024

Quick facts

Lead sponsorMaastricht University
StatusCompleted
Study typeOBSERVATIONAL
Enrollment7
Start date1 July 2020
Primary completion18 July 2024
Estimated completion18 July 2024
Sites1 location across Netherlands

Drugs / interventions tested

Conditions studied

Sponsor

Maastricht University

Who can join

18 and older, any sex, with MDC1A. Patients with the condition only — healthy volunteers not accepted.

Sponsor's own description

Merosin-deficient congenital muscle dystrophy type 1a (MDC1a), or LAMA2 muscular dystrophy (LAMA2-MD) is a severe autosomal recessive form of muscular dystrophy that is caused by homozygous or compound heterozygous mutations in the laminin alpha 2 (LAMA-2) gene. Many different LAMA-2 mutations have been reported. In most cases, MDC1a is diagnosed within the first year of life, and is characterized by hypotonia, delayed motor development and white matter abnormalities. Currently, no efficient treatment is available for this patient group. Generally, MDC1a patients with mutations causing a premature stop codon are most severely affected (early onset LAMA2-MD) and patients with missense mutations are generally affected more mild affected and more late-onset (late onset LAMA2-MD). However, large variation in disease severity and clinical course is observed, even between individuals with the same mutation, e.g. the LAMA2 c.5562+5G\>C mutation, which is frequently observed in Dutch MDC1a patients. This study aims to isolate and culture fibroblasts and myogenic stem cells called mesoangioblasts from the skin and muscle biopsies of adult LAMA2 mutation carriers to explore if genetic correction of LAMA2 mutations using CRISPR-Cas9 can be achieved and subsequently assess the effect in vitro, as a first step towards therapy development.

Publications & conference data

No peer-reviewed publications indexed yet for this trial. Completed trials usually publish results within 12-18 months.

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Other Maastricht University trials

Trials by the same sponsor.

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Data sources for this page

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