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Neurogenesis and neuronal migration in the forebrain of the TorsinA knockout mouse embryo.

Title: Neurogenesis and neuronal migration in the forebrain of the TorsinA knockout mouse embryo.
Name(s): McCarthy, Deirdre M, author
Gioioso, Valeria, author
Zhang, Xuan, author
Sharma, Nutan, author
Bhide, Pradeep G, author
Type of Resource: text
Genre: Journal Article
Date Issued: 2012-01-01
Physical Form: computer
online resource
Extent: 1 online resource
Language(s): English
Abstract/Description: Early-onset generalized torsion dystonia, also known as DYT1 dystonia, is a childhood onset heritable neurological movement disorder involving painful, involuntary muscle contractions, sustained abnormal postures, and repetitive movements. It is caused by a GAG deletion in the Tor1A gene located on chromosome 9. TorsinA, the product of the Tor1A gene, is expressed throughout the brain beginning early in embryonic development. It plays a role in the regulation of nuclear envelope-cytoskeletal interactions, and presumably nuclear translocation. Since nuclear translocation, powered by cytoskeletal traction, is critical for cell proliferation and migration, we examined whether neurogenesis and neuronal migration are affected in Tor1A-/- mouse brain. Our data show that interkinetic nuclear migration and the pattern of migration of newly generated neurons are impaired in the dorsal forebrain of the Tor1A-/- embryo. However, neurogenesis is not altered significantly. The rate of migration of cells from explants of the medial ganglionic eminence is also impaired in the Tor1A-/- embryo. Thus, loss of torsinA results in subtle but significant alterations in cell proliferation and migration in the embryonic forebrain. These subtle developmental changes are consistent with a lack of significant changes in neuronal numbers, neuronal positioning or size of brain regions in DYT1 dystonia patients.
Identifier: FSU_pmch_23018676 (IID), 10.1159/000342260 (DOI), PMC3712350 (PMCID), 23018676 (RID), 23018676 (EID), 000342260 (PII)
Grant Number: P50 NS037409, P30 NS045776, P30NS045776, R21 NS065266, P01 NS037409
Publication Note: This NIH-funded author manuscript originally appeared in PubMed Central at
Subject(s): Animals
Cell Count
Cell Division
Cell Movement/genetics
Cell Movement/physiology
Cell Shape
Dystonia Musculorum Deformans/embryology
Dystonia Musculorum Deformans/genetics
Dystonia Musculorum Deformans/pathology
Frontal Lobe/embryology
Frontal Lobe/pathology
Gene Expression Regulation, Developmental
Genes, Lethal
Gestational Age
Mice, Knockout
Molecular Chaperones/genetics
Molecular Chaperones/physiology
Nerve Tissue Proteins/biosynthesis
Nerve Tissue Proteins/deficiency
Nerve Tissue Proteins/genetics
Nerve Tissue Proteins/physiology
Nuclear Proteins/biosynthesis
Nuclear Proteins/genetics
Prefrontal Cortex/embryology
Prefrontal Cortex/pathology
RNA, Messenger/biosynthesis
RNA, Messenger/genetics
Transcription Factors/biosynthesis
Transcription Factors/genetics
Persistent Link to This Record:
Owner Institution: FSU
Is Part Of: Developmental neuroscience.
Issue: iss. 4, vol. 34

Choose the citation style.
McCarthy, D. M., Gioioso, V., Zhang, X., Sharma, N., & Bhide, P. G. (2012). Neurogenesis and neuronal migration in the forebrain of the TorsinA knockout mouse embryo. Developmental Neuroscience. Retrieved from