Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Fragile X mice develop sensory hyperreactivity to auditory stimuli.

L Chen1, M Toth

  • 1Department of Pharmacology, Weill Medical College of Cornell University, 1300 York Avenue, LC 522, New York, NY 10021, USA.

Neuroscience
|April 13, 2001
PubMed
Summary

Fragile X syndrome, a leading cause of intellectual disability, is linked to sensory hypersensitivity. Fragile X mice show abnormal auditory processing and heightened startle responses, offering a model for studying this condition.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Covalent linkage between proteins of the inter-alpha-inhibitor family and hyaluronic acid is mediated by a factor produced by granulosa cells.

The Journal of biological chemistry·1996
Same author

Two-tone rate suppression boundaries of cochlear ganglion neurons in normal chickens.

The Journal of the Acoustical Society of America·1996
Same author

Greater ozone-induced inflammatory responses in subjects with asthma.

American journal of respiratory and critical care medicine·1996
Same author

Use of combinatorial peptide libraries to construct functional mimics of tumor epitopes recognized by MHC class I-restricted cytolytic T lymphocytes.

The Journal of experimental medicine·1996
Same author

Recurrent transition at a CG dinucleotide in exon 12 of COL2A1 produces kniest dysplasia with abnormal RNA splicing by chondrocytes and lymphoblasts and interruption of the triple helix of type II collagen.

Annals of the New York Academy of Sciences·1996
Same author

Inogatran, a novel direct low molecular weight thrombin inhibitor, given with, but not after, tissue-plasminogen activator, improves thrombolysis.

The Journal of pharmacology and experimental therapeutics·1996

Area of Science:

  • Neuroscience
  • Genetics
  • Developmental Biology

Background:

  • Fragile X syndrome is the most common inherited cause of intellectual disability.
  • It results from the inactivation of the FMR-1 gene.
  • Beyond cognitive deficits, patients exhibit behavioral issues including sensory hypersensitivity.

Purpose of the Study:

  • To investigate sensory processing abnormalities in fragile X mice.
  • To determine if fragile X mice exhibit abnormal sensitivity to auditory stimuli.

Main Methods:

  • Utilized the prepulse inhibition paradigm to assess auditory startle responses.
  • Examined seizure susceptibility to auditory stimulation.
  • Investigated c-Fos expression in brain regions following auditory stimuli.

Related Experiment Videos

Main Results:

  • Fragile X mice displayed hyperreactivity to auditory stimuli in the prepulse inhibition test.
  • These mice showed increased susceptibility to audiogenic seizures.
  • Seizures involved auditory brainstem and thalamic nuclei, not general hyperexcitability.

Conclusions:

  • Fragile X mice exhibit auditory hypersensitivity, mirroring symptoms in human patients.
  • This auditory hypersensitivity suggests abnormal auditory system processing.
  • Fragile X mice serve as a valuable model for studying fragile X syndrome's neural basis.