Literature DB >> 27376585

Large-scale phenome analysis defines a behavioral signature for Huntington's disease genotype in mice.

Vadim Alexandrov1, Dani Brunner1, Liliana B Menalled1, Andrea Kudwa1, Judy Watson-Johnson1, Matthew Mazzella1, Ian Russell1, Melinda C Ruiz1, Justin Torello1, Emily Sabath1, Ana Sanchez1, Miguel Gomez1, Igor Filipov1, Kimberly Cox1, Mei Kwan1, Afshin Ghavami1, Sylvie Ramboz1, Brenda Lager2, Vanessa C Wheeler3, Jeff Aaronson2, Jim Rosinski2, James F Gusella3, Marcy E MacDonald3, David Howland2, Seung Kwak2.   

Abstract

Rapid technological advances for the frequent monitoring of health parameters have raised the intriguing possibility that an individual's genotype could be predicted from phenotypic data alone. Here we used a machine learning approach to analyze the phenotypic effects of polymorphic mutations in a mouse model of Huntington's disease that determine disease presentation and age of onset. The resulting model correlated variation across 3,086 behavioral traits with seven different CAG-repeat lengths in the huntingtin gene (Htt). We selected behavioral signatures for age and CAG-repeat length that most robustly distinguished between mouse lines and validated the model by correctly predicting the repeat length of a blinded mouse line. Sufficient discriminatory power to accurately predict genotype required combined analysis of >200 phenotypic features. Our results suggest that autosomal dominant disease-causing mutations could be predicted through the use of subtle behavioral signatures that emerge in large-scale, combinatorial analyses. Our work provides an open data platform that we now share with the research community to aid efforts focused on understanding the pathways that link behavioral consequences to genetic variation in Huntington's disease.

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Year:  2016        PMID: 27376585     DOI: 10.1038/nbt.3587

Source DB:  PubMed          Journal:  Nat Biotechnol        ISSN: 1087-0156            Impact factor:   54.908


  41 in total

1.  Cognitive deficits in transgenic and knock-in HTT mice parallel those in Huntington's disease.

Authors:  Andrew M Farrar; Carol A Murphy; Neil E Paterson; Stephen Oakeshott; Dansha He; William Alosio; Kristi McConnell; Liliana B Menalled; Sylvie Ramboz; Larry C Park; David Howland; Dani Brunner
Journal:  J Huntingtons Dis       Date:  2014

2.  Studies on neurotransmitter markers and striatal neuronal cell density in Huntington's disease and dentatorubropallidoluysian atrophy.

Authors:  I Kanazawa; H Sasaki; O Muramoto; M Matsushita; T Mizutani; K Iwabuchi; T Ikeda; N Takahata
Journal:  J Neurol Sci       Date:  1985-09       Impact factor: 3.181

3.  Mutation size and age at onset in Huntington's disease.

Authors:  D Craufurd; A Dodge
Journal:  J Med Genet       Date:  1993-12       Impact factor: 6.318

4.  A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease.

Authors: 
Journal:  Neurology       Date:  2001-08-14       Impact factor: 9.910

Review 5.  Mouse ENU mutagenesis.

Authors:  M J Justice; J K Noveroske; J S Weber; B Zheng; A Bradley
Journal:  Hum Mol Genet       Date:  1999       Impact factor: 6.150

6.  Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats.

Authors:  Liliana B Menalled; Jessica D Sison; Ioannis Dragatsis; Scott Zeitlin; Marie-Françoise Chesselet
Journal:  J Comp Neurol       Date:  2003-10-06       Impact factor: 3.215

7.  Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study.

Authors: 
Journal:  Arch Neurol       Date:  2008-12

8.  Systematic comparison of phenome-wide association study of electronic medical record data and genome-wide association study data.

Authors:  Joshua C Denny; Lisa Bastarache; Marylyn D Ritchie; Robert J Carroll; Raquel Zink; Jonathan D Mosley; Julie R Field; Jill M Pulley; Andrea H Ramirez; Erica Bowton; Melissa A Basford; David S Carrell; Peggy L Peissig; Abel N Kho; Jennifer A Pacheco; Luke V Rasmussen; David R Crosslin; Paul K Crane; Jyotishman Pathak; Suzette J Bielinski; Sarah A Pendergrass; Hua Xu; Lucia A Hindorff; Rongling Li; Teri A Manolio; Christopher G Chute; Rex L Chisholm; Eric B Larson; Gail P Jarvik; Murray H Brilliant; Catherine A McCarty; Iftikhar J Kullo; Jonathan L Haines; Dana C Crawford; Daniel R Masys; Dan M Roden
Journal:  Nat Biotechnol       Date:  2013-12       Impact factor: 54.908

9.  Circadian Abnormalities in Motor Activity in a BAC Transgenic Mouse Model of Huntington's Disease.

Authors:  Stephen Oakeshott; Fuat Balci; Igor Filippov; Carol Murphy; Russell Port; David Connor; Ahmad Paintdakhi; Joseph Lesauter; Liliana Menalled; Sylvie Ramboz; Seung Kwak; David Howland; Rae Silver; Dani Brunner
Journal:  PLoS Curr       Date:  2011-04-05

10.  Novel targets for Huntington's disease in an mTOR-independent autophagy pathway.

Authors:  Andrea Williams; Sovan Sarkar; Paul Cuddon; Evangelia K Ttofi; Shinji Saiki; Farah H Siddiqi; Luca Jahreiss; Angeleen Fleming; Dean Pask; Paul Goldsmith; Cahir J O'Kane; Rodrigo Andres Floto; David C Rubinsztein
Journal:  Nat Chem Biol       Date:  2008-05       Impact factor: 15.040

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  21 in total

1.  Deep representation learning of electronic health records to unlock patient stratification at scale.

Authors:  Isotta Landi; Benjamin S Glicksberg; Hao-Chih Lee; Sarah Cherng; Giulia Landi; Matteo Danieletto; Joel T Dudley; Cesare Furlanello; Riccardo Miotto
Journal:  NPJ Digit Med       Date:  2020-07-17

Review 2.  Progress in developing transgenic monkey model for Huntington's disease.

Authors:  Brooke R Snyder; Anthony W S Chan
Journal:  J Neural Transm (Vienna)       Date:  2017-11-10       Impact factor: 3.575

Review 3.  Close encounters: Moving along bumps, breaks, and bubbles on expanded trinucleotide tracts.

Authors:  Aris A Polyzos; Cynthia T McMurray
Journal:  DNA Repair (Amst)       Date:  2017-06-09

4.  Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington's disease.

Authors:  Robert M Bragg; Sydney R Coffey; Rory M Weston; Seth A Ament; Jeffrey P Cantle; Shawn Minnig; Cory C Funk; Dominic D Shuttleworth; Emily L Woods; Bonnie R Sullivan; Lindsey Jones; Anne Glickenhaus; John S Anderson; Michael D Anderson; Stephen B Dunnett; Vanessa C Wheeler; Marcy E MacDonald; Simon P Brooks; Nathan D Price; Jeffrey B Carroll
Journal:  Sci Rep       Date:  2017-02-08       Impact factor: 4.379

5.  Deep phenotyping predicts Huntington's genotype.

Authors:  Douglas M Ruderfer; Joel T Dudley
Journal:  Nat Biotechnol       Date:  2016-08-09       Impact factor: 54.908

6.  Single-Nucleus RNA-Seq Reveals Dysregulation of Striatal Cell Identity Due to Huntington's Disease Mutations.

Authors:  Sonia Malaiya; Marcia Cortes-Gutierrez; Brian R Herb; Sydney R Coffey; Samuel R W Legg; Jeffrey P Cantle; Carlo Colantuoni; Jeffrey B Carroll; Seth A Ament
Journal:  J Neurosci       Date:  2021-05-19       Impact factor: 6.167

7.  Genetic Contributors to Intergenerational CAG Repeat Instability in Huntington's Disease Knock-In Mice.

Authors:  João Luís Neto; Jong-Min Lee; Ali Afridi; Tammy Gillis; Jolene R Guide; Stephani Dempsey; Brenda Lager; Isabel Alonso; Vanessa C Wheeler; Ricardo Mouro Pinto
Journal:  Genetics       Date:  2016-12-02       Impact factor: 4.562

8.  MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in mice.

Authors:  Peter Langfelder; Fuying Gao; Nan Wang; David Howland; Seung Kwak; Thomas F Vogt; Jeffrey S Aaronson; Jim Rosinski; Giovanni Coppola; Steve Horvath; X William Yang
Journal:  PLoS One       Date:  2018-01-11       Impact factor: 3.240

9.  An Automated Home-Cage System to Assess Learning and Performance of a Skilled Motor Task in a Mouse Model of Huntington's Disease.

Authors:  Cameron L Woodard; Federico Bolaños; James D Boyd; Gergely Silasi; Timothy H Murphy; Lynn A Raymond
Journal:  eNeuro       Date:  2017-09-18

10.  Suppression of MAPK11 or HIPK3 reduces mutant Huntingtin levels in Huntington's disease models.

Authors:  Meng Yu; Yuhua Fu; Yijiang Liang; Haikun Song; Yao Yao; Peng Wu; Yuwei Yao; Yuyin Pan; Xue Wen; Lixiang Ma; Saiyin Hexige; Yu Ding; Shouqing Luo; Boxun Lu
Journal:  Cell Res       Date:  2017-10-13       Impact factor: 25.617

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