Skip to main content

This is a preprint.

It has not yet been peer reviewed by a journal.

The National Library of Medicine is running a pilot to include preprints that result from research funded by NIH in PMC and PubMed.

medRxiv logoLink to medRxiv
[Preprint]. 2024 May 3:2024.05.02.24306523. [Version 1] doi: 10.1101/2024.05.02.24306523

Long-read sequencing unravels the complexity of structural variants in PRKN in two individuals with early-onset Parkinson’s disease

Guillaume Cogan, Kensuke Daida, Kimberley J Billingsley, Christelle Tesson, Sylvie Forlani, Ludmila Jornea, Lionel Arnaud, Laurene Tissier, Eric LeGuern, Andrew B Singleton, Mélanie Ferrien, Hélène Gervais Bernard, Suzanne Lesage, Cornelis Blauwendraat, Alexis Brice
PMCID: PMC11092742  PMID: 38746197

Abstract

Background

PRKN biallelic pathogenic variants are the most common cause of autosomal recessive early-onset Parkinson’s disease (PD). However, the variants responsible for suspected PRKN- PD individuals are not always identified with standard genetic testing.

Objectives

Identify the genetic cause in two siblings with a PRKN -PD phenotype using long-read sequencing (LRS).

Methods

The genetic investigation involved standard testing using successively multiple ligation probe amplification (MLPA), Sanger sequencing, targeted sequencing, whole-exome sequencing and LRS.

Results

MLPA and targeted sequencing identified one copy of exon four in PRKN but no other variants were identified. Subsequently, LRS unveiled a large deletion encompassing exon 3 to 4 on one allele and a duplication of exon 3 on the second allele; explaining the siblings’ phenotype. MLPA could not identify the balanced rearrangement of exon 3.

Conclusions

This study highlights the potential utility of long-read sequencing in the context of unsolved typical PRKN- PD individuals.

Full Text Availability

The license terms selected by the author(s) for this preprint version do not permit archiving in PMC. The full text is available from the preprint server.


Articles from medRxiv are provided here courtesy of Cold Spring Harbor Laboratory Preprints

RESOURCES