Human de novo mutation rates from a four-generation pedigree reference

D David Porubsky H Harriet Dashnow T Thomas A. Sasani G Glennis A. Logsdon P Pille Hallast M Michelle D. Noyes Z Zev N. Kronenberg T Tom Mokveld N Nidhi Koundinya C Cillian Nolan C Cody J. Steely A Andrea Guarracino E Egor Dolzhenko W William T. Harvey W William J. Rowell K Kirill Grigorev T Thomas J. Nicholas M Michael E. Goldberg K Keisuke K. Oshima J Jiadong Lin P Peter Ebert W W. Scott Watkins T Tiffany Y. Leung V Vincent C. T. Hanlon S Sean McGee B Brent S. Pedersen H Hannah C. Happ H Hyeonsoo Jeong K Katherine M. Munson K Kendra Hoekzema D Daniel D. Chan Y Yanni Wang J Jordan Knuth G Gage H. Garcia C Cairbre Fanslow C Christine Lambert C Charles Lee J Joshua D. Smith S Shawn Levy C Christopher E. Mason E Erik Garrison P Peter M. Lansdorp D Deborah W. Neklason L Lynn B. Jorde A Aaron R. Quinlan M Michael A. Eberle E Evan E. Eichler

Abstract

Abstract Understanding the human de novo mutation (DNM) rate requires complete sequence information1. Here using five complementary short-read and long-read sequencing technologies, we phased and assembled more than 95% of each diploid human genome in a four-generation, twenty-eight-member family (CEPH 1463). We estimate 98–206 DNMs per transmission, including 74.5 de novo single-nucleotide variants, 7.4 non-tandem repeat indels, 65.3 de novo indels or structural variants originating from tandem repeats, and 4.4 centromeric DNMs. Among male individuals, we find 12.4 de novo Y chromosome events per generation. Short tandem repeats and variable-number tandem repeats are the most mutable, with 32 loci exhibiting recurrent mutation through the generations. We accurately assemble 288 centromeres and six Y chromosomes across the generations and demonstrate that the DNM rate varies by an order of magnitude depending on repeat content, length and sequence identity. We show a strong paternal bias (75–81%) for all forms of germline DNM, yet we estimate that 16% of de novo single-nucleotide variants are postzygotic in origin with no paternal bias, including early germline mosaic mutations. We place all this variation in the context of a high-resolution recombination map (~3.4 kb breakpoint resolution) and find no correlation between meiotic crossover and de novo structural variants. These near-telomere-to-telomere familial genomes provide a truth set to understand the most fundamental processes underlying human genetic variation.

Article Details

Journal Nature
Volume / Issue Vol. 643, Issue 8071
Published July 10, 2025
Pages 427-436
ISSN 0028-0836
Publisher Nature Portfolio

Journal Info

Nature

Nature Portfolio

ISSN: 0028-0836 Health Sciences

Authors (47)

D

David Porubsky

H

Harriet Dashnow

T

Thomas A. Sasani

G

Glennis A. Logsdon

P

Pille Hallast

M

Michelle D. Noyes

Z

Zev N. Kronenberg

T

Tom Mokveld

N

Nidhi Koundinya

C

Cillian Nolan

C

Cody J. Steely

A

Andrea Guarracino

E

Egor Dolzhenko

W

William T. Harvey

W

William J. Rowell

K

Kirill Grigorev

T

Thomas J. Nicholas

M

Michael E. Goldberg

K

Keisuke K. Oshima

J

Jiadong Lin

P

Peter Ebert

W

W. Scott Watkins

T

Tiffany Y. Leung

V

Vincent C. T. Hanlon

S

Sean McGee

B

Brent S. Pedersen

H

Hannah C. Happ

H

Hyeonsoo Jeong

K

Katherine M. Munson

K

Kendra Hoekzema

D

Daniel D. Chan

Y

Yanni Wang

J

Jordan Knuth

G

Gage H. Garcia

C

Cairbre Fanslow

C

Christine Lambert

C

Charles Lee

J

Joshua D. Smith

S

Shawn Levy

C

Christopher E. Mason

E

Erik Garrison

P

Peter M. Lansdorp

D

Deborah W. Neklason

L

Lynn B. Jorde

A

Aaron R. Quinlan

M

Michael A. Eberle

E

Evan E. Eichler