Activation of the ISR mediates the behavioral and neurophysiological abnormalities in Down syndrome-Reference-Cited by-同舟云学术

Activation of the ISR mediates the behavioral and neurophysiological abnormalities in Down syndrome

Published:2019-11-15 Issue:6467 Volume:366 Page:843-849
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

Zhu Ping Jun¹²^ORCID,Khatiwada Sanjeev¹²³^ORCID,Cui Ya⁴⁵^ORCID,Reineke Lucas C.¹²,Dooling Sean W.²⁶^ORCID,Kim Jean J.⁴⁷^ORCID,Li Wei⁴⁵,Walter Peter⁸⁹^ORCID,Costa-Mattioli Mauro¹²^ORCID

Affiliation:

1. Department of Neuroscience, Baylor College of Medicine, Houston, TX, USA.

2. Memory and Brain Research Center, Baylor College of Medicine, Houston, TX, USA.

3. Verna and Marrs McLean Department of Biochemistry and Molecular Biology, Baylor College of Medicine, Houston, TX, USA.

4. Division of Biostatistics, Dan L Duncan Comprehensive Cancer Center, and Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX, USA.

5. Department of Biological Chemistry, University of California, Irvine, Irvine, CA, USA.

6. Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA.

7. Stem Cells and Regenerative Medicine Center, Baylor College of Medicine, Houston, TX, USA.

8. Howard Hughes Medical Institute, University of California at San Francisco, San Francisco, CA, USA.

9. Department of Biochemistry and Biophysics, University of California at San Francisco, San Francisco, CA, USA.

Abstract

Tuning stress protects cognition Down syndrome (DS) is a chromosomal disorder that occurs when a person has an extra copy of chromosome 21. DS causes intellectual disabilities, among other health issues, but little is known about the mechanisms underlying the memory deficits in DS. Zhu et al. used a multidisciplinary approach to show that a defect in integrated stress response, a conserved pathway that controls protein homeostasis, can explain the cognitive and neuronal deficits in a mouse model of DS (see the Perspective by Halliday and Mallucci). These insights into the biological basis underlying DS could potentially help in the design of treatments for this condition. Science , this issue p. 843 ; see also p. 797

Funder

Howard Hughes Medical Institute

National Institute of Neurological Disorders and Stroke

Sammons Enterprises

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference68 articles.

1. Down Syndrome: From Understanding the Neurobiology to Therapy

2. Array-CGH detection of micro rearrangements in mentally retarded individuals: clinical significance of imbalances present both in affected children and normal parents

3. Genetic and Epigenetic Networks in Intellectual Disabilities