The Human Gene Mutation Database (HGMD®): optimizing its use in a clinical diagnostic or research setting

PD Stenson, M Mort, EV Ball, M Chapman, K Evans… - Human genetics, 2020 - Springer
Abstract The Human Gene Mutation Database (HGMD®) constitutes a comprehensive
collection of published germline mutations in nuclear genes that are thought to underlie, or …

Diverse molecular mechanisms underlying pathogenic protein mutations: beyond the loss-of-function paradigm

L Backwell, JA Marsh - Annual review of genomics and human …, 2022 - annualreviews.org
Most known disease-causing mutations occur in protein-coding regions of DNA. While some
of these involve a loss of protein function (eg, through premature stop codons or missense …

Loss-of-function, gain-of-function and dominant-negative mutations have profoundly different effects on protein structure

L Gerasimavicius, BJ Livesey, JA Marsh - Nature communications, 2022 - nature.com
Most known pathogenic mutations occur in protein-coding regions of DNA and change the
way proteins are made. Taking protein structure into account has therefore provided great …

The polygenic and monogenic basis of blood traits and diseases

D Vuckovic, EL Bao, P Akbari, CA Lareau, A Mousas… - Cell, 2020 - cell.com
Blood cells play essential roles in human health, underpinning physiological processes
such as immunity, oxygen transport, and clotting, which when perturbed cause a significant …

Pre-clinical study of induced pluripotent stem cell-derived dopaminergic progenitor cells for Parkinson's disease

D Doi, H Magotani, T Kikuchi, M Ikeda… - Nature …, 2020 - nature.com
Induced pluripotent stem cell (iPSC)-derived dopaminergic (DA) neurons are an expected
source for cell-based therapies for Parkinson's disease (PD). The regulatory criteria for the …

Integrated proteogenomic characterization across major histological types of pediatric brain cancer

F Petralia, N Tignor, B Reva, M Koptyra, S Chowdhury… - Cell, 2020 - cell.com
We report a comprehensive proteogenomics analysis, including whole-genome sequencing,
RNA sequencing, and proteomics and phosphoproteomics profiling, of 218 tumors across 7 …

[HTML][HTML] Targeted disruption of HLA genes via CRISPR-Cas9 generates iPSCs with enhanced immune compatibility

H Xu, BO Wang, M Ono, A Kagita, K Fujii, N Sasakawa… - Cell stem cell, 2019 - cell.com
Induced pluripotent stem cells (iPSCs) have strong potential in regenerative medicine
applications; however, immune rejection caused by HLA mismatching is a concern. B2M …

[HTML][HTML] Genomic basis for RNA alterations in cancer

PCAWG Transcriptome Core Group Calabrese Claudia … - Nature, 2020 - nature.com
Transcript alterations often result from somatic changes in cancer genomes. Various forms of
RNA alterations have been described in cancer, including overexpression, altered splicing …

Wilson disease

A Członkowska, T Litwin, P Dusek, P Ferenci… - Nature reviews Disease …, 2018 - nature.com
Wilson disease (WD) is a potentially treatable, inherited disorder of copper metabolism that
is characterized by the pathological accumulation of copper. WD is caused by mutations in …