Document Detail


Robinow syndrome.
MedLine Citation:
PMID:  12011143     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In 1969, Robinow and colleagues described a syndrome of mesomelic shortening, hemivertebrae, genital hypoplasia, and "fetal facies". Over 100 cases have now been reported and we have reviewed the current knowledge of the clinical and genetic features of the syndrome. The gene for the autosomal recessive form was identified as the ROR2 gene on chromosome 9q22. ROR2 is a receptor tyrosine kinase with orthologues in mouse and other species. The same gene, ROR2, has been shown to cause autosomal dominant brachydactyly B, but it is not known at present whether the autosomal dominant form of Robinow syndrome is also caused by mutations in ROR2.
Authors:
M A Patton; A R Afzal
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Journal of medical genetics     Volume:  39     ISSN:  1468-6244     ISO Abbreviation:  J. Med. Genet.     Publication Date:  2002 May 
Date Detail:
Created Date:  2002-05-15     Completed Date:  2002-05-28     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  2985087R     Medline TA:  J Med Genet     Country:  England    
Other Details:
Languages:  eng     Pagination:  305-10     Citation Subset:  IM    
Affiliation:
Department of Medical Genetics, St George's Hospital Medical School, Cranmer Terrace, London SW17 0RE, UK.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis*,  genetics*,  pathology
Animals
Arm / abnormalities,  radiography
Dwarfism / diagnosis*,  genetics*,  pathology
Facies
Genotype
Humans
Infant, Newborn
Male
Mice
Mutation, Missense
Penis / abnormalities
Phenotype
Rats
Receptor Tyrosine Kinase-like Orphan Receptors
Receptors, Cell Surface / genetics
Ribs / abnormalities,  radiography
Spinal Cord / abnormalities,  radiography
Syndrome
Chemical
Reg. No./Substance:
0/ROR2 protein, human; 0/Receptors, Cell Surface; EC 2.7.10.1/Receptor Tyrosine Kinase-like Orphan Receptors
Comments/Corrections

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