Document Detail


The molecular basis of the congenital fibrosis syndromes.
MedLine Citation:
PMID:  12221491     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The congenital fibrosis syndromes (CFS) are rare inherited strabismus syndromes that present as congenital, nonprogressive ophthalmoplegias with active and passive restriction of globe movement, and include classic and atypical forms of congenital fibrosis of the extraocular muscles (CFEOM) and Duane syndrome (DS). Although historically believed to result from primary extraocular muscle fibrosis, both neuropathologic and genetic studies support the hypothesis that these disorders result from aberrant development of motor nuclei in the midbrain and pons.
Authors:
Elizabeth C Engle
Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Strabismus     Volume:  10     ISSN:  0927-3972     ISO Abbreviation:  Strabismus     Publication Date:  2002 Jun 
Date Detail:
Created Date:  2002-09-10     Completed Date:  2002-11-26     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  9310896     Medline TA:  Strabismus     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  125-8     Citation Subset:  IM    
Affiliation:
Children's Hospital and Harvard Medical School, Boston, MA 02115, USA. engle@enders.tch.harvard.edu
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MeSH Terms
Descriptor/Qualifier:
Blepharoptosis / genetics
DNA / analysis
Eye Diseases, Hereditary / genetics*
Fibrosis / congenital
Homeodomain Proteins / genetics
Humans
Oculomotor Muscles / pathology*
Ophthalmoplegia / congenital*,  genetics*
Pedigree
Strabismus / genetics
Syndrome
Grant Support
ID/Acronym/Agency:
EY12498/EY/NEI NIH HHS; EY13583/EY/NEI NIH HHS; P30 HD18655/HD/NICHD NIH HHS
Chemical
Reg. No./Substance:
0/Homeodomain Proteins; 0/PHOX2A protein, human; 9007-49-2/DNA

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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