Genotype to phenotype-discovery and characterization of novel genomic disorders in a "genotype-first" era

Genet Med. 2009 Dec;11(12):836-42. doi: 10.1097/GIM.0b013e3181c175d2.

Abstract

Recent advances in technology for detecting copy number changes have enabled genome-wide detection of submicroscopic deletions and duplications. As these technologies are applied to large cohorts of patients with diverse phenotypes, novel genomic disorders are defined by a common genotype (deletion or duplication) rather than a common phenotype. The discovery of new genomic disorders using this "genotype-first" approach has increased dramatically, and several recently described recurrent rearrangements are associated with a surprisingly wide range of phenotypes. This review will discuss the importance of genomic architecture for generating recurrent rearrangements, implications of the genotype-first approach for medical genetics, and features of several new genomic disorders with highly variable phenotypes.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Chromosome Aberrations*
  • Chromosome Deletion
  • Chromosome Disorders / genetics*
  • Chromosome Disorders / pathology
  • Gene Duplication
  • Gene Rearrangement
  • Genome, Human / genetics*
  • Genomics / methods*
  • Genomics / trends
  • Genotype
  • Humans
  • Phenotype