Document Detail


Norrie disease: linkage analysis using a 4.2-kb RFLP detected by a human ornithine aminotransferase cDNA probe.
MedLine Citation:
PMID:  2568328     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Previous study has shown that the usual DNA marker for Norrie disease, the L1.28 probe which identifies the DXS7 locus, can recombine with the disease locus. In this study, we used a human ornithine aminotransferase (OAT) cDNA which detects OAT-related DNA sequences mapped to the same region on the X chromosome as that of the L1.28 probe to investigate the family with Norrie disease who exhibited the recombinational event. When genomic DNA from this family was digested with the PvuII restriction endonuclease, we found a restriction fragment length polymorphism (RFLP) of 4.2 kb in size. This fragment was absent in the affected males and cosegregated with the disease locus; we calculated a lod score of 0.602, at theta = 0.00. No deletion could be detected by chromosomal analysis or on Southern blots with other enzymes. These results suggest that one of the OAT-related sequences on the X chromosome may be in close proximity to the Norrie disease locus and represent the first report which indicates that the OAT cDNA may be useful for the identification of carrier status and/or prenatal diagnosis.
Authors:
J T Ngo; J B Bateman; V Cortessis; R S Sparkes; T Mohandas; G Inana; M A Spence
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Genomics     Volume:  4     ISSN:  0888-7543     ISO Abbreviation:  Genomics     Publication Date:  1989 May 
Date Detail:
Created Date:  1989-08-22     Completed Date:  1989-08-22     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  8800135     Medline TA:  Genomics     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  539-45     Citation Subset:  IM    
Affiliation:
Jules Stein Eye Institute, UCLA School of Medicine 90024.
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MeSH Terms
Descriptor/Qualifier:
Animals
Blindness / enzymology,  genetics*
DNA
DNA Probes*
Female
Genetic Markers
Humans
Hybrid Cells
Lod Score
Male
Mice
Ornithine-Oxo-Acid Transaminase / genetics*
Polymorphism, Genetic*
Polymorphism, Restriction Fragment Length*
Recombination, Genetic
Retina / abnormalities*
Transaminases / genetics*
X Chromosome*
Grant Support
ID/Acronym/Agency:
EY-06772/EY/NEI NIH HHS; EY-07026/EY/NEI NIH HHS
Chemical
Reg. No./Substance:
0/DNA Probes; 0/Genetic Markers; 9007-49-2/DNA; EC 2.6.1.-/Transaminases; EC 2.6.1.13/Ornithine-Oxo-Acid Transaminase

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


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