Journal article
Sex-specific effects of the Huntington gene on normal neurodevelopment
Journal of neuroscience research, Vol.95(1-2), pp.398-408
01/02/2017
DOI: 10.1002/jnr.23980
PMCID: PMC5729280
PMID: 27870408
Abstract
Huntington disease is a neurodegenerative disorder caused by a gene (HTT) with a unique feature of trinucleotide repeats ranging from 10 to 35 in healthy people; when expanded beyond 39 repeats, Huntington disease develops. Animal models demonstrate that HTT is vital to brain development; however, this has not been studied in humans. Moreover, evidence suggests that triplet repeat genes may have been vital in evolution of the human brain. Here we evaluate brain structure using magnetic resonance imaging and brain function using cognitive tests in a sample of school-aged children ages 6 to 18 years old. DNA samples were processed to quantify the number of CAG repeats within HTT. We find that the number of repeats in HTT, below disease threshold, confers advantageous changes in brain structure and general intelligence (IQ): the higher the number of repeats, the greater the change in brain structure, and the higher the IQ. The pattern of structural brain changes associated with HTT is strikingly different between males and females. HTT may confer an advantage or a disadvantage depending on the repeat length, playing a key role in either the evolution of a superior human brain or development of a uniquely human brain disease. © 2016 Wiley Periodicals, Inc.
Details
- Title: Subtitle
- Sex-specific effects of the Huntington gene on normal neurodevelopment
- Creators
- Jessica K Lee - Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IowaYue Ding - Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IowaAmy L Conrad - Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IowaElena Cattaneo - Department of Biosciences, University of Milan, Milan, ItalyEric Epping - Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IowaKathy Mathews - Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, IowaPedro Gonzalez-Alegre - Department of Neurology, University of Pennsylvania Perelman School of Medicine, Philadelphia, PennsylvaniaLarry Cahill - Department of Neurobiology and Behavior, University of California, Irvine, CaliforniaVincent Magnotta - Department of Radiology, University of Iowa Carver College of Medicine, Iowa City, IowaBradley L Schlaggar - Department of Psychiatry, Washington University School of Medicine, St. Louis, MissouriJoel S Perlmutter - Department of Neurology, Washington University School of Medicine, St. Louis, MissouriRegina E Y Kim - Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IowaJeffrey D Dawson - Department of Biostatistics, College of Public Health, University of Iowa, Iowa City, IowaPeg Nopoulos - Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, Iowa
- Resource Type
- Journal article
- Publication Details
- Journal of neuroscience research, Vol.95(1-2), pp.398-408
- DOI
- 10.1002/jnr.23980
- PMID
- 27870408
- PMCID
- PMC5729280
- NLM abbreviation
- J Neurosci Res
- ISSN
- 0360-4012
- eISSN
- 1097-4547
- Publisher
- United States
- Grant note
- U54 TR001013 / NCATS NIH HHS U54 TR001356 / NCATS NIH HHS R01 NS055903 / NINDS NIH HHS
- Language
- English
- Date published
- 01/02/2017
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Public Health Administration; Psychiatry; Pediatric Psychology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Biostatistics; Craniofacial Anomalies Research Center; Neurology (Pediatrics)
- Record Identifier
- 9983997445902771
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