Centers for Mendelian Genomics: A decade of facilitating gene discovery

SM Baxter, JE Posey, NJ Lake, N Sobreira… - Genetics in …, 2022 - Elsevier
Purpose Mendelian disease genomic research has undergone a massive transformation
over the past decade. With increasing availability of exome and genome sequencing, the …

Targeted sequencing approach and its clinical applications for the molecular diagnosis of human diseases

XM Pei, MHY Yeung, ANN Wong, HF Tsang, ACS Yu… - Cells, 2023 - mdpi.com
The outbreak of COVID-19 has positively impacted the NGS market recently. Targeted
sequencing (TS) has become an important routine technique in both clinical and research …

De novo variants in the RNU4-2 snRNA cause a frequent neurodevelopmental syndrome

Y Chen, R Dawes, HC Kim, A Ljungdahl, SL Stenton… - Nature, 2024 - nature.com
Around 60% of individuals with neurodevelopmental disorders (NDD) remain undiagnosed
after comprehensive genetic testing, primarily of protein-coding genes. Large genome …

A deep intronic splice–altering AIRE variant causes APECED syndrome through antisense oligonucleotide-targetable pseudoexon inclusion

S Ochoa, AP Hsu, AJ Oler, D Kumar… - Science translational …, 2024 - science.org
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a life-
threatening monogenic autoimmune disorder primarily caused by biallelic deleterious …

Seven years since the launch of the Matchmaker Exchange: The evolution of genomic matchmaking

KM Boycott, DR Azzariti, A Hamosh… - Human mutation, 2022 - Wiley Online Library
Abstract The Matchmaker Exchange (MME) was launched in 2015 to provide a robust
mechanism to discover novel disease‐gene relationships. It operates as a federated …

Multi-omics identifies large mitoribosomal subunit instability caused by pathogenic MRPL39 variants as a cause of pediatric onset mitochondrial disease

SSC Amarasekera, DH Hock, NJ Lake… - Human molecular …, 2023 - academic.oup.com
MRPL39 encodes one of 52 proteins comprising the large subunit of the mitochondrial
ribosome (mitoribosome). In conjunction with 30 proteins in the small subunit, the …

Genomics 2 Proteins portal: a resource and discovery tool for linking genetic screening outputs to protein sequences and structures

S Kwon, J Safer, DT Nguyen, D Hoksza, P May… - Nature …, 2024 - nature.com
Recent advances in AI-based methods have revolutionized the field of structural biology.
Concomitantly, high-throughput sequencing and functional genomics have generated …

[HTML][HTML] Bi-allelic loss-of-function variants in WBP4, encoding a spliceosome protein, result in a variable neurodevelopmental syndrome

E Engal, KT Oja, R Maroofian, O Geminder… - The American Journal of …, 2023 - cell.com
Over two dozen spliceosome proteins are involved in human diseases, also referred to as
spliceosomopathies. WW domain-binding protein 4 (WBP4) is part of the early spliceosomal …

Exome sequencing and the identification of new genes and shared mechanisms in polymicrogyria

SK Akula, AY Chen, JE Neil, DD Shao, A Mo… - JAMA …, 2023 - jamanetwork.com
Importance Polymicrogyria is the most commonly diagnosed cortical malformation and is
associated with neurodevelopmental sequelae including epilepsy, motor abnormalities, and …

Critical assessment of variant prioritization methods for rare disease diagnosis within the Rare Genomes Project

SL Stenton, MC O'Leary, G Lemire, GE VanNoy… - Human Genomics, 2024 - Springer
Background A major obstacle faced by families with rare diseases is obtaining a genetic
diagnosis. The average" diagnostic odyssey" lasts over five years and causal variants are …