Tyrosyl-tRNA sequestration triggers tyrosyl-tRNA synthetase-associated peripheral neuropathy.
Paper
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The authors' code
R · 120 lines · 3.9 KB · MIT
- ### script to calibrate reads based on manual inspection
- # load libs
- library(foreach)
- library(doParallel)
- source('functions.R')
- # load parameters
- conf <- load_mapping_config()
- # load tool locations
- tool_path <- load_tool_location()
- # set max cores
- max_cores <- get_max_cores() # from functions.R
- # register max 8 cores because filtering process is I/O intense (mainly read and writing)
- if(max_cores > 8) {
- max_cores <- 8
- }
- registerDoParallel(makeCluster(max_cores))
- # get args
- get_dir <- commandArgs(trailingOnly = TRUE)
- # check input dir
- in_path <- parse_dir(get_dir[1])
- if(!dir.exists(in_path)){
- stop(
- paste0(in_path, ' does not exist!')
- )
- }
- # check if bam files
- in_files <- list.files(
- path = in_path,
- pattern = '.sort.bam',
- ignore.case = TRUE
- )
- if(length(in_files) < 1){
- stop('No BAM files found!')
- }
- # check config for calibration
- in_conf <- get_dir[2]
- if(grep('.csv$',in_conf) != 1){
- stop(paste0(in_conf, ' is not a CSV file!'))
- }
- # check if 5prime or 3 prime calibration
- if(length(grep('5prime',in_conf)) == 1){
- firstLast <- 'first'
- } else if(length(grep('3prime',in_conf)) == 1){
- firstLast <- 'last'
- } else {
- stop('No "5prime" or "3prime" in the filename of the config file for read calibraton found. Script
- does not known whether to perform 5\' or 3\'. Please keep the string "5prime" or "3prime"
- filename \n')
- }
- # read and check
- cali_conf <- read.table(in_conf, header = T, stringsAsFactors = F, sep = ',')
- if(nrow(cali_conf) < 1){
- stop(paste0(in_conf, ' has no rows! No read length processed!'))
- }
- in_f <- gsub(".sort.bam","",in_files)
- in_f2 <- in_f[is.element(in_f,colnames(cali_conf))]
- cat(paste0(
- length(in_f), ' / ', length(in_f),' found in calibration config file! \n'
- ))
- # check output dir
- out_path <- parse_dir(get_dir[3])
- if(!dir.exists(out_path)){
- dir.create(out_path)
- }
- if(!dir.exists(paste0(out_path,'calibration/'))){
- dir.create(paste0(out_path,'calibration/'))
- }
- if(!dir.exists(paste0(out_path,'calibration/calibrated/'))){
- dir.create(paste0(out_path,'calibration/calibrated/'))
- }
- # build vec for parallel exec
- par_files <- rep(in_f2, each = nrow(cali_conf) )
- par_read_length <- rep(cali_conf[,1], length(in_f2))
- ### split by length and take first nucleotide only
- cat('Calibrating reads by length and determined nucleotide position ... ')
- for_res <- foreach(i = 1:length(par_files)) %dopar% {
- # get nucleotide to take
- nt <- cali_conf[cali_conf[,1]==par_read_length[i],colnames(cali_conf)==par_files[i]]
- # 5' or 3' calibration
- if(firstLast == 'first'){
- f_read <- paste0("print $1 \"\t\" $2 \"\t\" $3 \"\t\" ($4-1+",nt,"+1) \"\t\" $5 \"\t1M\t\" $7 \"\t\" $8 \"\t\" $9 \"\t\" substr($10,(",nt,"+1),1) \"\t\" substr($11,(",nt,"+1),1); ")
- r_read <- paste0("print $1 \"\t\" $2 \"\t\" $3 \"\t\" ($4+length($10)-",nt,"-1) \"\t\" $5 \"\t1M\t\" $7 \"\t\" $8 \"\t\" $9 \"\t\" substr($10,(length($10)+1-",nt,"-1),1) \"\t\" substr($11,(length($11)+1-",nt,"-1),1); ")
- } else if(firstLast == 'last') {
- r_read <- paste0("print $1 \"\t\" $2 \"\t\" $3 \"\t\" ($4-1+",nt,"+1) \"\t\" $5 \"\t1M\t\" $7 \"\t\" $8 \"\t\" $9 \"\t\" substr($10,(",nt,"+1),1) \"\t\" substr($11,(",nt,"+1),1); ")
- f_read <- paste0("print $1 \"\t\" $2 \"\t\" $3 \"\t\" ($4+length($10)-",nt,"-1) \"\t\" $5 \"\t1M\t\" $7 \"\t\" $8 \"\t\" $9 \"\t\" substr($10,(length($10)+1-",nt,"-1),1) \"\t\" substr($11,(length($11)+1-",nt,"-1),1); ")
- }
- # check if NA or > 0
- if(!is.na(nt) && !is.null(nt) && nt > 0){
- # run
- system(
- paste0(
- tool_path['samtools'],' view -h ',
- in_path,par_files[i],'.sort.bam',
- " | awk '$1 ~ /@/ || length($10) == ",par_read_length[i],"'",
- " | awk '{
- if($1 ~ /@/)
- print $0;
- else if($2 == 0)
- ",f_read,"
- else if($2 == 16)
- ",r_read,"
- }'",
- ' | ',tool_path['samtools'],' view -S -b - > ',
- out_path,'calibration/calibrated/',gsub(".sort.bam","",par_files[i]),'_',par_read_length[i],'_calibrated.bam'
- )
- )
- }
- }
- cat('DONE \n')
calibrate_reads.R at commit f0751f3, under MIT · at the source
Overview
- Molecular Neurobiology Laboratory, Donders Institute for Brain, Cognition and Behaviour and Faculty of Science, Radboud University, Nijmegen 6525 AJ, The Netherlands
- The Jackson Laboratory, Bar Harbor, ME 04609, USA
- Biochemistry and Molecular Biology, Department of Chemistry, University of Hamburg, Hamburg 20146, Germany
- Jerry R. Mendell Center for Gene Therapy, The Abigail Wexner Research Institute at Nationwide Children’s Hospital, Columbus, OH 43202, USA
- Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH 43201, USA
Abstract
Mono-allelic mutations in seven cytosolic aminoacyl-tRNA synthetase genes cause Charcot–Marie–Tooth (CMT) peripheral neuropathy.
Here, we report that tyrosyl-tRNA (tRNATyr) sequestration by CMT-mutant tyrosyl-tRNA synthetase (TyrRS) is the molecular root cause of CMT-YARS1. tRNATyr sequestration depletes the cellular free tRNATyr pool, leading to insufficient tyrosyl-tRNATyr production and ribosome stalling at tyrosine codons.
In three Drosophila CMT-YARS1 models, transgenic overexpression of tRNATyr rescued peripheral neuropathy phenotypes and defective mRNA translation. We provide biochemical evidence for tRNATyr sequestration by the three CMT-TyrRS variants expressed in the Drosophila models and ribosome profiling revealed selective ribosome pausing at the two tyrosine codons. In the Yars1E196K mouse model, adeno-associated virus serotype 9 (AAV9)-mediated overexpression of tRNATyr—but not wild-type TyrRS protein—fully rescued motor performance deficits, reduced nerve conduction velocity and reduced motor axon calibre. Furthermore, AAV9-tRNATyr gene therapy prevented activation of the integrated stress response in spinal motor neurons, indicating resolution of ribosome stalling.
Thus, elevating tRNATyr levels constitutes a novel therapeutic approach for CMT-YARS1.
Reproduced under the paper's license (CC BY), from the paper cited above.
Repository
Its files are read in the Code ↔ Paper reader above.
AlexanderBartholomaeus/MiMB_ribosome_profiling
f0751f30d91d10de0281ae98a49724c4f21c2ff9, 7 April 2020Availability: 1 check, the latest on 5 November 2026: the link answers
- 5 November 2026: the link answers
16 files
- calibrate_reads.R, R, 120 lines
- calibration_count_plot.R
, R, 208 lines - coverage_start_stop.R, R, 148 lines
- filter_length.R, R, 83 lines
- functions.R, R, 126 lines
- gff2bed.R, R, 64 lines
- highest_expressed_genes.
R , R, 85 lines - install_R_packages.R, R, 10 lines
- mapping.R, R, 128 lines
- merge_reads.R, R, 71 lines
- prepare_coverage.R, R, 76 lines
- read_length_distribution
.R , R, 103 lines - split_by_length.R, R, 144 lines
- verify_dependencies.R, R, 40 lines
- LICENSE, License, 21 lines
- README.md, Text, 160 lines
Tracing map
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Data
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Data availability
Ribosome profiling data were deposited in the Gene Omnibus (GEO) under the accession number GSE268213. Any newly generated materials, including DNA constructs and fly lines reported in this paper, are available from the corresponding author () upon request.
Reproduced under the paper's license (CC BY), from the paper cited above.
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Version 1, 5 November 2026: the first record
Recorded: type, language, journal, volume, issue, pages, dates, 16 authors, 6 keywords, 13 MeSH terms, 16 funders, 0 references.
Cite
This paper
Mallik, M., Tadenev, A. L. D., Mora, N., Lewandowski, V., Köster, D., Landínez-Macías, M., Thompson, R., Menafra, M. P., Taylor, N. K., van Lith, P., van Bakel, N., Catinozzi, M., Harper, S. Q., Ignatova, Z., Burgess, R. W., & Storkebaum, E. (2026). Tyrosyl-tRNA sequestration triggers tyrosyl-tRNA synthetase-associated peripheral neuropathy. Brain : a journal of neurology, 149(11), 4052-4065. https://
BibTeX
@article{mallik2026tyros
author = {Mallik, Moushami and Tadenev, Abigail L D and Mora, Natalia and Lewandowski, Vanessa and Köster, Daniel and Landínez-Macías, María and Thompson, Robin and Menafra, María P and Taylor, Noah K and van Lith, Pascal and van Bakel, Nick and Catinozzi, Marica and Harper, Scott Q and Ignatova, Zoya and Burgess, Robert W and Storkebaum, Erik},
title = {{Tyrosyl-tRNA sequestration triggers tyrosyl-tRNA synthetase-associated peripheral neuropathy}},
journal = {Brain : a journal of neurology},
year = {2026},
month = nov,
volume = {149},
number = {11},
pages = {4052--4065},
publisher = {Oxford University Press},
issn = {0006-8950},
doi = {10.1093/
url = {https://
pmid = {42703926},
pmcid = {PMC13730582}
}
RIS
TY - JOUR
AU - Mallik, Moushami
AU - Tadenev, Abigail L D
AU - Mora, Natalia
AU - Lewandowski, Vanessa
AU - Köster, Daniel
AU - Landínez-Macías, María
AU - Thompson, Robin
AU - Menafra, María P
AU - Taylor, Noah K
AU - van Lith, Pascal
AU - van Bakel, Nick
AU - Catinozzi, Marica
AU - Harper, Scott Q
AU - Ignatova, Zoya
AU - Burgess, Robert W
AU - Storkebaum, Erik
TI - Tyrosyl-tRNA sequestration triggers tyrosyl-tRNA synthetase-associated peripheral neuropathy
T2 - Brain : a journal of neurology
J2 - Brain
PY - 2026
DA - 2026/
VL - 149
IS - 11
SP - 4052
EP - 4065
SN - 0006-8950
PB - Oxford University Press
DO - 10.1093/
UR - https://
LA - en
ER -
CSL-JSON
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"title": "Tyrosyl-tRNA sequestration triggers tyrosyl-tRNA synthetase-associated peripheral neuropathy",
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