The mutational constraint spectrum quantified from variation in 141,456 humans

Спектр мутационных ограничений, количественно оценённый на основе вариаций у 141 456 человек
Leif Groop, Dana Dabelea, Mark I. McCarthy, Olle Melander, Peter M. Nilsson, Stacey Gabriel, Gad Getz, Kristian Cibulskis, Jeff Gentry, Harlan M. Krumholz, Diego Ardissino, Terho Lehtimäki, Heribert Schunkert, Jaakko Kaprio, Judy H. Cho, Mina K. Chung, John Barnard, Kyong Soo Park, Michael Boehnke, Steven A. McCarroll, Daniel I. Chasman, Benjamin M. Neale, Mark J. Daly, James B. Meigs, Josée Dupuis, Eric Banks, Monkol Lek, Konrad J. Karczewski, Eric Vallabh Minikel, Kaitlin E. Samocha, Anne O’Donnell‐Luria, James S. Ware, Beryl B. Cummings, Daniel P. Birnbaum, Jack A. Kosmicki, Emma Pierce‐Hoffman, Laura D. Gauthier, Namrata Gupta, Lorena Orozco, Valentín Ruano-Rubio, Grace Tiao, Ben Weisburd, Stacey Donnelly, Roberto Elosúa, José C. Florez, Stephen J. Glatt, Christina M. Hultman, Sekar Kathiresan, Markku Laakso, Dermot McGovern, Ruth McPherson, Aarno Palotie, Danish Saleheen, Jeremiah M. Scharf, Pamela Sklar, Patrick F. Sullivan, Ming T. Tsuang, Hugh Watkins, James G. Wilson, Daniel G. MacArthur, Michael O‘Donovan, Hilkka Soininen, Mikko Hiltunen, Andres Metspalu, Carlos A. Aguilar‐Salinas, Patrick T. Ellinor, Steven A. Lubitz, Emelia J. Benjamin, Juliana C.N. Chan, Dan M. Roden, Jaume Marrugat, John D. Rioux, Erkki Vartiainen, Ryan L. Collins, Harrison Brand, Michael E. Talkowski, Nazneen Rahman, Lori L. Bonnycastle, Döst Öngür, André Franke, Jeanette Erdmann, Ramnik J. Xavier, Rinse K. Weersma, Tim D. Spector, Jessica Alföldi, Yik Ying Teo, Jaspal S. Kooner, Gil Atzmon, Soo Heon Kwak, Ronald C.W., Andrea Saltzman, Erwin P. Böttinger, Miguel Covarrubias, Raymond K. Walters, Andrea Ganna, Jessica X. Chong, Ruth J. F. Loos, John C. Chambers, E Shyong Tai, Donald W. Bowden, Ravindranath Duggirala, Christine M. Albert, Charlotte Tolonen, Tõnu Esko, Dawood Darbar, Matthew J. Bown, Tariq Ahmad, Harry Sokol, Martti Färkkilâ, Benjamin Gläser, Irina M. Armean, Jaana Suvisaari, Laurent C. Francioli, Nicholas A. Watts, Matthew Solomonson, Ruchi Munshi, Kristen M. Laricchia, Louis Bergelson, Kristen M. Connolly, Yossi Farjoun, Steven Ferriera, Thibault Jeandet, Diane Kaplan, Christopher Llanwarne, Sam Novod, Nikelle Petrillo, Timothy Poterba, David Roazen, Molly Schleicher, Cotton Seed, José Soto, Kathleen Tibbetts, Christopher Vittal, Gordon Wade, Arcturus Wang, Qingbo S. Wang, Nicola Whiffin, Laurent Beaugerie, Bruce M. Cohen, Daniel R. Rhodes, Moriel Singer‐Berk, Eleina England, Eleanor G. Seaby, Katherine Tashman, Zachary Zappala, Adolfo Correa, David Goldstein, Clicerio González, George Kirov, Dan Turner, Craig L. Hanis, Young Jin Kim, Teresa Tusié‐Luna, Veikko Salomaa, Colin N. A. Palmer, Marquis P. Vawter, Michael J. Owen, Christopher Haiman, Matti Holi, Mikko Kallela, Bong-Jo Kim, Seppo Koskinen, Kari M. Mattila, Carlos N. Pato, Anne M. Remes, Samuli Ripatti, M. Benjamin Shoemaker, Tuomi Tiinamaija, Maija Wessman, Matthew Harms, Subra Kugathasan, Ann E. Pulver, Nilesh J. Samani, Stacey Gabriel
2020-05-27

Genome Aggregation Databasegene discoveryhuman protein-coding genesmutational constraintpredicted loss-of-function variants
Abstract Genetic variants that inactivate protein-coding genes are a powerful source of information about the phenotypic consequences of gene disruption: genes that are crucial for the function of an organism will be depleted of such variants in natural populations, whereas non-essential genes will tolerate their accumulation. However, predicted loss-of-function variants are enriched for annotation errors, and tend to be found at extremely low frequencies, so their analysis requires careful variant annotation and very large sample sizes 1 . Here we describe the aggregation of 125,748 exomes and 15,708 genomes from human sequencing studies into the Genome Aggregation Database (gnomAD). We identify 443,769 high-confidence predicted loss-of-function variants in this cohort after filtering for artefacts caused by sequencing and annotation errors. Using an improved model of human mutation rates, we classify human protein-coding genes along a spectrum that represents tolerance to inactivation, validate this classification using data from model organisms and engineered human cells, and show that it can be used to improve the power of gene discovery for both common and rare diseases.
1
After filtering sequencing and annotation artefacts, the cohort contains 443,769 high-confidence predicted loss-of-function variants.
2
An improved human mutation-rate model classifies protein-coding genes along a spectrum of tolerance to inactivation.
3
Gene intolerance classifications are validated using model-organism data and engineered human cells.
4
The constraint spectrum improves gene-discovery power for both common and rare diseases.
5
The study aggregates 125,748 exomes and 15,708 genomes into the Genome Aggregation Database (gnomAD).

human protein-coding genes and their predicted loss-of-function variation in 141,456 sequenced humans

the spectrum of gene tolerance to inactivation, including depletion of loss-of-function variants and its implications for gene discovery

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Publication Date
2020-05-27
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Authors
Leif Groop
Dana Dabelea
Mark I. McCarthy
Olle Melander
Peter M. Nilsson
Stacey Gabriel
Gad Getz
Kristian Cibulskis
Jeff Gentry
Harlan M. Krumholz
Diego Ardissino
Terho Lehtimäki
Heribert Schunkert
Jaakko Kaprio
Judy H. Cho
Mina K. Chung
John Barnard
Kyong Soo Park
Michael Boehnke
Steven A. McCarroll
Daniel I. Chasman
Benjamin M. Neale
Mark J. Daly
James B. Meigs
Josée Dupuis
Eric Banks
Monkol Lek
Konrad J. Karczewski
Eric Vallabh Minikel
Kaitlin E. Samocha
Anne O’Donnell‐Luria
James S. Ware
Beryl B. Cummings
Daniel P. Birnbaum
Jack A. Kosmicki
Emma Pierce‐Hoffman
Laura D. Gauthier
Namrata Gupta
Lorena Orozco
Valentín Ruano-Rubio
Grace Tiao
Ben Weisburd
Stacey Donnelly
Roberto Elosúa
José C. Florez
Stephen J. Glatt
Christina M. Hultman
Sekar Kathiresan
Markku Laakso
Dermot McGovern
Ruth McPherson
Aarno Palotie
Danish Saleheen
Jeremiah M. Scharf
Pamela Sklar
Patrick F. Sullivan
Ming T. Tsuang
Hugh Watkins
James G. Wilson
Daniel G. MacArthur
Michael O‘Donovan
Hilkka Soininen
Mikko Hiltunen
Andres Metspalu
Carlos A. Aguilar‐Salinas
Patrick T. Ellinor
Steven A. Lubitz
Emelia J. Benjamin
Juliana C.N. Chan
Dan M. Roden
Jaume Marrugat
John D. Rioux
Erkki Vartiainen
Ryan L. Collins
Harrison Brand
Michael E. Talkowski
Nazneen Rahman
Lori L. Bonnycastle
Döst Öngür
André Franke
Jeanette Erdmann
Ramnik J. Xavier
Rinse K. Weersma
Tim D. Spector
Jessica Alföldi
Yik Ying Teo
Jaspal S. Kooner
Gil Atzmon
Soo Heon Kwak
Ronald C.W.
Andrea Saltzman
Erwin P. Böttinger
Miguel Covarrubias
Raymond K. Walters
Andrea Ganna
Jessica X. Chong
Ruth J. F. Loos
John C. Chambers
E Shyong Tai
Donald W. Bowden
Ravindranath Duggirala
Christine M. Albert
Charlotte Tolonen
Tõnu Esko
Dawood Darbar
Matthew J. Bown
Tariq Ahmad
Harry Sokol
Martti Färkkilâ
Benjamin Gläser
Irina M. Armean
Jaana Suvisaari
Laurent C. Francioli
Nicholas A. Watts
Matthew Solomonson
Ruchi Munshi
Kristen M. Laricchia
Louis Bergelson
Kristen M. Connolly
Yossi Farjoun
Steven Ferriera
Thibault Jeandet
Diane Kaplan
Christopher Llanwarne
Sam Novod
Nikelle Petrillo
Timothy Poterba
David Roazen
Molly Schleicher
Cotton Seed
José Soto
Kathleen Tibbetts
Christopher Vittal
Gordon Wade
Arcturus Wang
Qingbo S. Wang
Nicola Whiffin
Laurent Beaugerie
Bruce M. Cohen
Daniel R. Rhodes
Moriel Singer‐Berk
Eleina England
Eleanor G. Seaby
Katherine Tashman
Zachary Zappala
Adolfo Correa
David Goldstein
Clicerio González
George Kirov
Dan Turner
Craig L. Hanis
Young Jin Kim
Teresa Tusié‐Luna
Veikko Salomaa
Colin N. A. Palmer
Marquis P. Vawter
Michael J. Owen
Christopher Haiman
Matti Holi
Mikko Kallela
Bong-Jo Kim
Seppo Koskinen
Kari M. Mattila
Carlos N. Pato
Anne M. Remes
Samuli Ripatti
M. Benjamin Shoemaker
Tuomi Tiinamaija
Maija Wessman
Matthew Harms
Subra Kugathasan
Ann E. Pulver
Nilesh J. Samani
Stacey Gabriel
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