Quantifying prediction of pathogenicity for within-codon concordance (PM5) using 7541 functional classifications of BRCA1 and MSH2 missense variants.


Journal

Genetics in medicine : official journal of the American College of Medical Genetics
ISSN: 1530-0366
Titre abrégé: Genet Med
Pays: United States
ID NLM: 9815831

Informations de publication

Date de publication:
03 2022
Historique:
received: 04 08 2021
revised: 21 10 2021
accepted: 12 11 2021
pubmed: 16 12 2021
medline: 24 3 2022
entrez: 15 12 2021
Statut: ppublish

Résumé

Conditions and thresholds applied for evidence weighting of within-codon concordance (PM5) for pathogenicity vary widely between laboratories and expert groups. Because of the sparseness of available clinical classifications, there is little evidence for variation in practice. We used as a truthset 7541 dichotomous functional classifications of BRCA1 and MSH2, spanning 311 codons of BRCA1 and 918 codons of MSH2, generated from large-scale functional assays that have been shown to correlate excellently with clinical classifications. We assessed PM5 at 5 stringencies with incorporation of 8 in silico tools. For each analysis, we quantified a positive likelihood ratio (pLR, true positive rate/false positive rate), the predictive value of PM5-lookup in ClinVar compared with the functional truthset. pLR was 16.3 (10.6-24.9) for variants for which there was exactly 1 additional colocated deleterious variant on ClinVar, and the variant under examination was equally or more damaging when analyzed using BLOSUM62. pLR was 71.5 (37.8-135.3) for variants for which there were 2 or more colocated deleterious ClinVar variants, and the variant under examination was equally or more damaging than at least 1 colocated variant when analyzed using BLOSUM62. These analyses support the graded use of PM5, with potential to use it at higher evidence weighting where more stringent criteria are met.

Identifiants

pubmed: 34906453
pii: S1098-3600(21)05389-2
doi: 10.1016/j.gim.2021.11.011
pmc: PMC8896276
pii:
doi:

Substances chimiques

BRCA1 Protein 0
BRCA1 protein, human 0
Codon 0
MSH2 protein, human EC 3.6.1.3
MutS Homolog 2 Protein EC 3.6.1.3

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

552-563

Subventions

Organisme : Medical Research Council
ID : MC_UP_1102/20
Pays : United Kingdom
Organisme : Cancer Research UK
ID : 27223
Pays : United Kingdom
Organisme : Wellcome Trust
ID : 107469/Z/15/Z
Pays : United Kingdom
Organisme : Cancer Research UK
ID : C61296/A27223
Pays : United Kingdom
Organisme : British Heart Foundation
ID : RE/18/4/34215
Pays : United Kingdom
Organisme : Wellcome Trust
Pays : United Kingdom

Investigateurs

S Samant (S)
A Lucassen (A)
A Znaczko (A)
A Shaw (A)
A Ansari (A)
A Kumar (A)
A Donaldson (A)
A Murray (A)
A Ross (A)
A Taylor-Beadling (A)
A Taylor (A)
A Innes (A)
A Brady (A)
A Kulkarni (A)
A-C Hogg (AC)
A Ramsay Bowden (AR)
A Hadonou (A)
B Coad (B)
B McIldowie (B)
B Speight (B)
B DeSouza (B)
B Mullaney (B)
C McKenna (C)
C Brewer (C)
C Olimpio (C)
C Clabby (C)
C Crosby (C)
C Jenkins (C)
C Armstrong (C)
C Bowles (C)
C Brooks (C)
C Byrne (C)
C Maurer (C)
D Baralle (D)
D Chubb (D)
D Stobo (D)
D Moore (D)
D O'Sullivan (D)
D Donnelly (D)
D Randhawa (D)
D Halliday (D)
E Atkinson (E)
E Baple (E)
E Rauter (E)
E Johnston (E)
E Woodward (E)
E Maher (E)
E Sofianopoulou (E)
E Petrides (E)
F Lalloo (F)
F McRonald (F)
F Pelz (F)
I Frayling (I)
G Evans (G)
G Corbett (G)
G Rea (G)
H Clouston (H)
H Powell (H)
H Williamson (H)
H Carley (H)
H J W Thomas (HJW)
I Tomlinson (I)
J Cook (J)
J Hoyle (J)
J Tellez (J)
J Whitworth (J)
J Williams (J)
J Murray (J)
J Campbell (J)
J Tolmie (J)
J Field (J)
J Mason (J)
J Burn (J)
J Bruty (J)
J Callaway (J)
J Grant (J)
J Del Rey Jimenez (J)
J Pagan (J)
J VanCampen (J)
J Barwell (J)
K Monahan (K)
K Tatton-Brown (K)
K-R Ong (KR)
K Murphy (K)
K Andrews (K)
K Mokretar (K)
K Cadoo (K)
K Smith (K)
K Baker (K)
K Brown (K)
K Reay (K)
K McKay Bounford (K)
K Bradshaw (K)
K Russell (K)
K Stone (K)
K Snape (K)
L Crookes (L)
L Reed (L)
L Taggart (L)
L Yarram (L)
L Cobbold (L)
L Walker (L)
L Walker (L)
L Hawkes (L)
L Busby (L)
L Izatt (L)
L Kiely (L)
L Hughes (L)
L Side (L)
L Sarkies (L)
K-L Greenhalgh (KL)
M Shanmugasundaram (M)
M Duff (M)
M Bartlett (M)
M Watson (M)
M Owens (M)
M Bradford (M)
M Huxley (M)
M Slean (M)
M Ryten (M)
M Smith (M)
M Ahmed (M)
N Roberts (N)
C O'Brien (C)
O Middleton (O)
P Tarpey (P)
P Logan (P)
P Dean (P)
P May (P)
P Brace (P)
R Tredwell (R)
R Harrison (R)
R Hart (R)
R Kirk (R)
R Martin (R)
R Nyanhete (R)
R Wright (R)
R Martin (R)
R Davidson (R)
R Cleaver (R)
S Talukdar (S)
S Butler (S)
J Sampson (J)
S Ribeiro (S)
S Dell (S)
S Mackenzie (S)
S Hegarty (S)
S Albaba (S)
S McKee (S)
S Palmer-Smith (S)
S Heggarty (S)
S MacParland (S)
S Greville-Heygate (S)
S Daniels (S)
S Prapa (S)
S Abbs (S)
S Tennant (S)
S Hardy (S)
S MacMahon (S)
T McVeigh (T)
T Foo (T)
T Bedenham (T)
T Cranston (T)
T McDevitt (T)
V Clowes (V)
V Tripathi (V)
V McConnell (V)
N Woodwaer (N)
Y Wallis (Y)
Z Kemp (Z)
G Mullan (G)
L Pierson (L)
L Rainey (L)
C Joyce (C)
A Timbs (A)
A-M Reuther (AM)
B Frugtniet (B)
B DeSouza (B)
C Husher (C)
C Lawn (C)
C Corbett (C)
D Nocera-Jijon (D)
D Reay (D)
E Cross (E)
F Ryan (F)
H Lindsay (H)
J Oliver (J)
J Dring (J)
J Spiers (J)
J Harper (J)
K Ciucias (K)
L Connolly (L)
M Tsang (M)
R Brown (R)
S Shepherd (S)
S Begum (S)
S Daniels (S)
T Tadiso (T)
T Linton-Willoughby (T)
H Heppell (H)
K Sahan (K)
L Worrillow (L)
Z Allen (Z)
M Barlett (M)
C Watt (C)
M Hegarty (M)

Informations de copyright

Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Conflict of Interest The authors declare no conflicts of interest.

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Auteurs

Lucy Loong (L)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Cankut Cubuk (C)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Subin Choi (S)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Sophie Allen (S)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Beth Torr (B)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Alice Garrett (A)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Chey Loveday (C)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom.

Miranda Durkie (M)

Sheffield Diagnostic Genetics Service, NHS North East and Yorkshire Genomic Laboratory Hub, Sheffield Children's NHS Foundation Trust, Sheffield, United Kingdom.

Alison Callaway (A)

Wessex Regional Genetics Laboratory, Salisbury NHS Foundation Trust, Salisbury, United Kingdom; Human Genetics and Genomic Medicine, Faculty of Medicine, University of Southampton, Southampton, United Kingdom.

George J Burghel (GJ)

Manchester Centre for Genomic Medicine and North West Genomic Laboratory Hub, Manchester University NHS Foundation Trust, Manchester, United Kingdom.

James Drummond (J)

East Genomic Laboratory Hub, Cambridge University Hospitals Genomic Laboratory, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.

Rachel Robinson (R)

North East and Yorkshire Genomic Laboratory Hub, The Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom.

Ian R Berry (IR)

Bristol Genetics Laboratory, Pathology Sciences, Southmead Hospital, North Bristol NHS Trust, Bristol, United Kingdom.

Andrew Wallace (A)

Manchester Centre for Genomic Medicine and North West Genomic Laboratory Hub, Manchester University NHS Foundation Trust, Manchester, United Kingdom.

Diana M Eccles (DM)

Cancer Sciences, Faculty of Medicine, University of Southampton, Southampton, United Kingdom.

Marc Tischkowitz (M)

Department of Medical Genetics, NIHR Research Cambridge Biomedical Research Centre, University of Cambridge, Cambridge, United Kingdom.

Sian Ellard (S)

Department of Molecular Genetics, Royal Devon and Exeter NHS Foundation Trust, Exeter, United Kingdom.

James S Ware (JS)

National Heart and Lung Institute, Faculty of Medicine, and MRC London Institute of Medical Sciences, Imperial College London, London, United Kingdom; NIHR Royal Brompton Cardiovascular Research Centre, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom.

Helen Hanson (H)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom; Department of Clinical Genetics, St. George's University Hospitals NHS Foundation Trust, London, United Kingdom.

Clare Turnbull (C)

Division of Genetics and Epidemiology, The Institute of Cancer Research, Sutton, United Kingdom; Cancer Genetics Unit, The Royal Marsden NHS Foundation Trust, London, United Kingdom. Electronic address: clare.turnbull@icr.ac.uk.

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