A locus for ophthalmo-acromelic syndrome mapped to 10p11.23

Am J Med Genet A. 2009 Mar;149A(3):336-42. doi: 10.1002/ajmg.a.32656.

Abstract

Ophthalmo-acromelic syndrome (OAS, OMIM %206920) is a rare autosomal recessive disease, presenting with clinical anophthalmia and limb anomalies. We recruited three OAS families including a Japanese family with two affected patients and two consanguineous Lebanese families each having an affected. Homozygosity mapping was performed using the 50K SNP chip and additional informative markers. A locus for OAS was mapped to the 422-kb region at 10q11.23, based on the results from the two consanguineous families as well as the consistent data from the Japanese non-consanguineous family. The 422-kb region only contained one gene, MPP7. Although we could not detect any pathological mutations in OAS families analyzed, MPP7 could remain a candidate as aberrant changes might exist beyond our mutation detection methods. Further families are needed to confirm this candidate locus.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anophthalmos / genetics*
  • Brain / diagnostic imaging
  • Child
  • Child, Preschool
  • Chromosome Mapping*
  • Chromosomes, Human, Pair 10*
  • Consanguinity
  • DNA Mutational Analysis
  • Female
  • Genes, Recessive
  • Genetic Markers
  • Haplotypes
  • Homozygote
  • Humans
  • Limb Deformities, Congenital / genetics
  • Male
  • Membrane Proteins / genetics
  • Pedigree
  • Polymorphism, Single Nucleotide
  • Radiography
  • Siblings
  • Syndrome

Substances

  • Genetic Markers
  • MPP7 protein, human
  • Membrane Proteins