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Loss of NARS1 impairs progenitor proliferation in cortical brain organoids and leads to microcephaly
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Loss of NARS1 impairs progenitor proliferation in cortical brain organoids and leads to microcephaly
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Journal Article
Loss of NARS1 impairs progenitor proliferation in cortical brain organoids and leads to microcephaly
2020
Overview
Asparaginyl-tRNA synthetase1 (NARS1) is a member of the ubiquitously expressed cytoplasmic Class IIa family of tRNA synthetases required for protein translation. Here, we identify biallelic missense and frameshift mutations in
NARS1
in seven patients from three unrelated families with microcephaly and neurodevelopmental delay. Patient cells show reduced NARS1 protein, impaired NARS1 activity and impaired global protein synthesis. Cortical brain organoid modeling shows reduced proliferation of radial glial cells (RGCs), leading to smaller organoids characteristic of microcephaly. Single-cell analysis reveals altered constituents of both astrocytic and RGC lineages, suggesting a requirement for
NARS1
in RGC proliferation. Our findings demonstrate that
NARS1
is required to meet protein synthetic needs and to support RGC proliferation in human brain development.
Asparaginyl-tRNA synthetase1 (NARS1) is required for protein synthesis. Here, the authors identify biallelic NARS1 mutations in individuals with microcephaly and neurodevelopmental delay. Cortical brain organoid modeling recapitulates microcephaly characteristics and scRNA-seq reveals a role for NARS1 in radial glial cell proliferation.
Publisher
Nature Publishing Group UK,Nature Publishing Group,Nature Portfolio
Subject
/ 38/23
/ 45/77
/ 96
/ 96/2
/ Adult
/ Aspartate-tRNA Ligase - deficiency
/ Aspartate-tRNA Ligase - genetics
/ Brain
/ Child
/ Family
/ Female
/ Humanities and Social Sciences
/ Humans
/ Induced Pluripotent Stem Cells - metabolism
/ Male
/ Mutation
/ Neural Stem Cells - metabolism
/ Neural Stem Cells - pathology
/ Pedigree
/ Proteins
/ RNA, Transfer, Amino Acyl - genetics
/ Science
/ tRNA
