학술논문

Whole‐genome sequencing identifies EN1 as a determinant of bone density and fracture
Document Type
article
Author
Zheng, Hou‐FengForgetta, VincenzoHsu, Yi‐HsiangEstrada, KarolRosello‐Diez, AlbertoLeo, Paul JDahia, Chitra LPark‐Min, Kyung HyunTobias, Jonathan HKooperberg, CharlesKleinman, AaronStyrkarsdottir, UnnurLiu, Ching‐TiUggla, CharlottaEvans, Daniel SNielson, Carrie MWalter, KlaudiaPettersson‐Kymmer, UlrikaMcCarthy, ShaneEriksson, JoelKwan, TonyJhamai, MilaTrajanoska, KaterinaMemari, YasinMin, JosineHuang, JieDanecek, PetrWilmot, BethLi, RuiChou, Wen‐ChiMokry, Lauren EMoayyeri, AlirezaClaussnitzer, MelinaCheng, Chia‐HoCheung, WarrenMedina‐Gómez, CarolinaGe, BingChen, Shu‐HuangChoi, KwangbomOei, LingFraser, JamesKraaij, RobertHibbs, Matthew AGregson, Celia LPaquette, DenisHofman, AlbertWibom, CarlTranah, Gregory JMarshall, MhairiGardiner, Brooke BCremin, KatieAuer, PaulHsu, LiRing, SueTung, Joyce YThorleifsson, GudmarEnneman, Anke Wvan Schoor, Natasja Mde Groot, Lisette CPGMvan der Velde, NathalieMelin, BeatriceKemp, John PChristiansen, ClausSayers, AdrianZhou, YanhuaCalderari, Sophievan Rooij, JeroenCarlson, ChrisPeters, UlrikeBerlivet, SoizikDostie, JoséeUitterlinden, Andre GWilliams, Stephen RFarber, CharlesGrinberg, DanielLaCroix, Andrea ZHaessler, JeffChasman, Daniel IGiulianini, FrancoRose, Lynda MRidker, Paul MEisman, John ANguyen, Tuan VCenter, Jacqueline RNogues, XavierGarcia‐Giralt, NataliaLauner, Lenore LGudnason, VilmunderMellström, DanVandenput, LiesbethAmin, Najafvan Duijn, Cornelia MKarlsson, Magnus KLjunggren, ÖstenSvensson, OlleHallmans, GöranRousseau, FrançoisGiroux, SylvieBussière, JohanneArp, Pascal P
Source
Nature. 526(7571)
Subject
Biological Sciences
Biomedical and Clinical Sciences
Clinical Sciences
Genetics
Human Genome
Biotechnology
Osteoporosis
Stem Cell Research
2.1 Biological and endogenous factors
1.1 Normal biological development and functioning
Aetiology
Underpinning research
Musculoskeletal
Injuries and accidents
Animals
Bone Density
Bone and Bones
Disease Models
Animal
Europe
Exome
Female
Fractures
Bone
Gene Frequency
Genetic Predisposition to Disease
Genetic Variation
Genome
Human
Genomics
Genotype
Homeodomain Proteins
Humans
Mice
Sequence Analysis
DNA
White People
Wnt Proteins
AOGC Consortium
UK10K Consortium
General Science & Technology
Language
Abstract
The extent to which low-frequency (minor allele frequency (MAF) between 1-5%) and rare (MAF ≤ 1%) variants contribute to complex traits and disease in the general population is mainly unknown. Bone mineral density (BMD) is highly heritable, a major predictor of osteoporotic fractures, and has been previously associated with common genetic variants, as well as rare, population-specific, coding variants. Here we identify novel non-coding genetic variants with large effects on BMD (ntotal = 53,236) and fracture (ntotal = 508,253) in individuals of European ancestry from the general population. Associations for BMD were derived from whole-genome sequencing (n = 2,882 from UK10K (ref. 10); a population-based genome sequencing consortium), whole-exome sequencing (n = 3,549), deep imputation of genotyped samples using a combined UK10K/1000 Genomes reference panel (n = 26,534), and de novo replication genotyping (n = 20,271). We identified a low-frequency non-coding variant near a novel locus, EN1, with an effect size fourfold larger than the mean of previously reported common variants for lumbar spine BMD (rs11692564(T), MAF = 1.6%, replication effect size = +0.20 s.d., Pmeta = 2 × 10(-14)), which was also associated with a decreased risk of fracture (odds ratio = 0.85; P = 2 × 10(-11); ncases = 98,742 and ncontrols = 409,511). Using an En1(cre/flox) mouse model, we observed that conditional loss of En1 results in low bone mass, probably as a consequence of high bone turnover. We also identified a novel low-frequency non-coding variant with large effects on BMD near WNT16 (rs148771817(T), MAF = 1.2%, replication effect size = +0.41 s.d., Pmeta = 1 × 10(-11)). In general, there was an excess of association signals arising from deleterious coding and conserved non-coding variants. These findings provide evidence that low-frequency non-coding variants have large effects on BMD and fracture, thereby providing rationale for whole-genome sequencing and improved imputation reference panels to study the genetic architecture of complex traits and disease in the general population.