American Journal of Ophthalmology
Volume 149, Issue 5 , Pages 861-866, May 2010

A Novel Locus for Autosomal Recessive Retinitis Pigmentosa in a Consanguineous Pakistani Family Maps to Chromosome 2p

  • Shagufta Naz

      Affiliations

    • National Centre of Excellence in Molecular Biology, University of the Punjab, Lahore Pakistan
  • ,
  • S. Amer Riazuddin

      Affiliations

    • National Centre of Excellence in Molecular Biology, University of the Punjab, Lahore Pakistan
  • ,
  • Lin Li

      Affiliations

    • Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, Maryland
  • ,
  • Mariam Shahid

      Affiliations

    • National Centre of Excellence in Molecular Biology, University of the Punjab, Lahore Pakistan
  • ,
  • Samra Kousar

      Affiliations

    • National Centre of Excellence in Molecular Biology, University of the Punjab, Lahore Pakistan
  • ,
  • Paul A. Sieving

      Affiliations

    • Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, Maryland
  • ,
  • J. Fielding Hejtmancik

      Affiliations

    • Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, Maryland
  • ,
  • Sheikh Riazuddin

      Affiliations

    • National Centre of Excellence in Molecular Biology, University of the Punjab, Lahore Pakistan
    • Corresponding Author InformationInquiries to Sheikh Riazuddin, National Centre of Excellence in Molecular Biology, 87 West Canal Bank Road, Lahore 53700, Pakistan

Accepted 21 December 2009. published online 15 March 2010.

Objective

To identify a disease locus for autosomal recessive retinitis pigmentosa in a consanguineous Pakistani family.

Design

Prospective linkage study.

Methods

Blood samples were collected and genomic DNA was extracted. A genome-wide scan was performed using 382 polymorphic microsatellite markers on genomic DNA from 4 affected and 5 unaffected family members, and logarithm of odds scores were calculated.

Results

A maximum 2-point logarithm of odds score of 3.14 at θ = 0 was obtained for marker D2S165 during the genome-wide scan. Fine mapping markers identified a 20.92-cM (19.98-Mb) interval flanked by D2S149 and D2S367 that cosegregates with the disease phenotype. Haplotype analyses further refined the critical interval, distal to D2S220 in a 12.31-cM (13.35-Mb) region that does not harbor any genes that previously have been associated with retinitis pigmentosa.

Conclusions

Linkage analysis identified a new locus for autosomal recessive retinitis pigmentosa that maps to chromosome 2p22.3-p24.1 in a consanguineous Pakistani family.

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PII: S0002-9394(09)00975-1

doi:10.1016/j.ajo.2009.12.034

American Journal of Ophthalmology
Volume 149, Issue 5 , Pages 861-866, May 2010