Comprehensive Motor Testing in Fmr1-KO Mice Exposes Temporal Defects in Oromotor Coordination

Snigdha Roy, Yu Zhao, Melody Allensworth, Mohamed F. Farook, Mark S. LeDoux, Lawrence T. Reiter, Detlef H. Heck

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Fragile X syndrome (FXS; MIM #300624), a well-recognized form of inherited human mental retardation is caused, in most cases, by a CGG trinucleotide repeat expansion in the 5'-untranslated region of FMR1, resulting in reduced expression of the fragile X mental retardation protein (FMRP). Clinical features include macroorchidism, anxiety, mental retardation, motor coordination, and speech articulation deficits. The Fmr1 knockout (Fmr1-KO) mouse, a mouse model for FXS, has been shown to replicate the macroorchidism, cognitive deficits, and neuroanatomical abnormalities found in human FXS. Here we asked whether Fmr1-KO mice also display appendicular and oromotor deficits comparable to the ataxia and dysarthric speech seen in FXS patients. We employed standard motor tests for balance and appendicular motor coordination, and used a novel long-term fluid-licking assay to investigate oromotor function in Fmr1-KO mice and their wild-type (WT) littermates. Fmr1-KO mice performed equally well as their WT littermates on standard motor tests, with the exception of a raised-beam task. However, Fmr1-KO mice had a significantly slower licking rhythm than their WT littermates. Deficits in rhythmic fluid-licking in Fmr1-KO mice have been linked to cerebellar pathologies. It is believed that balance and motor coordination deficits in FXS patients are caused by cerebellar neurophathologies. The neuronal bases of speech articulation deficits in FXS patients are currently unknown. It is yet to be established whether similar neuronal circuits control rhythmic fluid-licking pattern in mice and speech articulation movement in humans.

Original languageEnglish (US)
Pages (from-to)962-969
Number of pages8
JournalBehavioral Neuroscience
Volume125
Issue number6
DOIs
StatePublished - Dec 1 2011

Fingerprint

Knockout Mice
Intellectual Disability
Fragile X Mental Retardation Protein
Trinucleotide Repeat Expansion
Fragile X Syndrome
5' Untranslated Regions
Ataxia
Anxiety
Pathology

All Science Journal Classification (ASJC) codes

  • Behavioral Neuroscience

Cite this

Comprehensive Motor Testing in Fmr1-KO Mice Exposes Temporal Defects in Oromotor Coordination. / Roy, Snigdha; Zhao, Yu; Allensworth, Melody; Farook, Mohamed F.; LeDoux, Mark S.; Reiter, Lawrence T.; Heck, Detlef H.

In: Behavioral Neuroscience, Vol. 125, No. 6, 01.12.2011, p. 962-969.

Research output: Contribution to journalArticle

Roy, Snigdha ; Zhao, Yu ; Allensworth, Melody ; Farook, Mohamed F. ; LeDoux, Mark S. ; Reiter, Lawrence T. ; Heck, Detlef H. / Comprehensive Motor Testing in Fmr1-KO Mice Exposes Temporal Defects in Oromotor Coordination. In: Behavioral Neuroscience. 2011 ; Vol. 125, No. 6. pp. 962-969.
@article{7a36f601253d4c4b9c03de850324f70d,
title = "Comprehensive Motor Testing in Fmr1-KO Mice Exposes Temporal Defects in Oromotor Coordination",
abstract = "Fragile X syndrome (FXS; MIM #300624), a well-recognized form of inherited human mental retardation is caused, in most cases, by a CGG trinucleotide repeat expansion in the 5'-untranslated region of FMR1, resulting in reduced expression of the fragile X mental retardation protein (FMRP). Clinical features include macroorchidism, anxiety, mental retardation, motor coordination, and speech articulation deficits. The Fmr1 knockout (Fmr1-KO) mouse, a mouse model for FXS, has been shown to replicate the macroorchidism, cognitive deficits, and neuroanatomical abnormalities found in human FXS. Here we asked whether Fmr1-KO mice also display appendicular and oromotor deficits comparable to the ataxia and dysarthric speech seen in FXS patients. We employed standard motor tests for balance and appendicular motor coordination, and used a novel long-term fluid-licking assay to investigate oromotor function in Fmr1-KO mice and their wild-type (WT) littermates. Fmr1-KO mice performed equally well as their WT littermates on standard motor tests, with the exception of a raised-beam task. However, Fmr1-KO mice had a significantly slower licking rhythm than their WT littermates. Deficits in rhythmic fluid-licking in Fmr1-KO mice have been linked to cerebellar pathologies. It is believed that balance and motor coordination deficits in FXS patients are caused by cerebellar neurophathologies. The neuronal bases of speech articulation deficits in FXS patients are currently unknown. It is yet to be established whether similar neuronal circuits control rhythmic fluid-licking pattern in mice and speech articulation movement in humans.",
author = "Snigdha Roy and Yu Zhao and Melody Allensworth and Farook, {Mohamed F.} and LeDoux, {Mark S.} and Reiter, {Lawrence T.} and Heck, {Detlef H.}",
year = "2011",
month = "12",
day = "1",
doi = "10.1037/a0025920",
language = "English (US)",
volume = "125",
pages = "962--969",
journal = "Behavioral Neuroscience",
issn = "0735-7044",
publisher = "American Psychological Association Inc.",
number = "6",

}

TY - JOUR

T1 - Comprehensive Motor Testing in Fmr1-KO Mice Exposes Temporal Defects in Oromotor Coordination

AU - Roy, Snigdha

AU - Zhao, Yu

AU - Allensworth, Melody

AU - Farook, Mohamed F.

AU - LeDoux, Mark S.

AU - Reiter, Lawrence T.

AU - Heck, Detlef H.

PY - 2011/12/1

Y1 - 2011/12/1

N2 - Fragile X syndrome (FXS; MIM #300624), a well-recognized form of inherited human mental retardation is caused, in most cases, by a CGG trinucleotide repeat expansion in the 5'-untranslated region of FMR1, resulting in reduced expression of the fragile X mental retardation protein (FMRP). Clinical features include macroorchidism, anxiety, mental retardation, motor coordination, and speech articulation deficits. The Fmr1 knockout (Fmr1-KO) mouse, a mouse model for FXS, has been shown to replicate the macroorchidism, cognitive deficits, and neuroanatomical abnormalities found in human FXS. Here we asked whether Fmr1-KO mice also display appendicular and oromotor deficits comparable to the ataxia and dysarthric speech seen in FXS patients. We employed standard motor tests for balance and appendicular motor coordination, and used a novel long-term fluid-licking assay to investigate oromotor function in Fmr1-KO mice and their wild-type (WT) littermates. Fmr1-KO mice performed equally well as their WT littermates on standard motor tests, with the exception of a raised-beam task. However, Fmr1-KO mice had a significantly slower licking rhythm than their WT littermates. Deficits in rhythmic fluid-licking in Fmr1-KO mice have been linked to cerebellar pathologies. It is believed that balance and motor coordination deficits in FXS patients are caused by cerebellar neurophathologies. The neuronal bases of speech articulation deficits in FXS patients are currently unknown. It is yet to be established whether similar neuronal circuits control rhythmic fluid-licking pattern in mice and speech articulation movement in humans.

AB - Fragile X syndrome (FXS; MIM #300624), a well-recognized form of inherited human mental retardation is caused, in most cases, by a CGG trinucleotide repeat expansion in the 5'-untranslated region of FMR1, resulting in reduced expression of the fragile X mental retardation protein (FMRP). Clinical features include macroorchidism, anxiety, mental retardation, motor coordination, and speech articulation deficits. The Fmr1 knockout (Fmr1-KO) mouse, a mouse model for FXS, has been shown to replicate the macroorchidism, cognitive deficits, and neuroanatomical abnormalities found in human FXS. Here we asked whether Fmr1-KO mice also display appendicular and oromotor deficits comparable to the ataxia and dysarthric speech seen in FXS patients. We employed standard motor tests for balance and appendicular motor coordination, and used a novel long-term fluid-licking assay to investigate oromotor function in Fmr1-KO mice and their wild-type (WT) littermates. Fmr1-KO mice performed equally well as their WT littermates on standard motor tests, with the exception of a raised-beam task. However, Fmr1-KO mice had a significantly slower licking rhythm than their WT littermates. Deficits in rhythmic fluid-licking in Fmr1-KO mice have been linked to cerebellar pathologies. It is believed that balance and motor coordination deficits in FXS patients are caused by cerebellar neurophathologies. The neuronal bases of speech articulation deficits in FXS patients are currently unknown. It is yet to be established whether similar neuronal circuits control rhythmic fluid-licking pattern in mice and speech articulation movement in humans.

UR - http://www.scopus.com/inward/record.url?scp=83055184741&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=83055184741&partnerID=8YFLogxK

U2 - 10.1037/a0025920

DO - 10.1037/a0025920

M3 - Article

C2 - 22004265

AN - SCOPUS:83055184741

VL - 125

SP - 962

EP - 969

JO - Behavioral Neuroscience

JF - Behavioral Neuroscience

SN - 0735-7044

IS - 6

ER -