Defining the genetic basis of early onset hereditary spastic paraplegia using whole genome sequencing

Neurogenetics. 2016 Oct;17(4):265-270. doi: 10.1007/s10048-016-0495-z. Epub 2016 Sep 28.

Abstract

We performed whole genome sequencing (WGS) in nine families from India with early-onset hereditary spastic paraplegia (HSP). We obtained a genetic diagnosis in 4/9 (44 %) families within known HSP genes (DDHD2 and CYP2U1), as well as perixosomal biogenesis disorders (PEX16) and GM1 gangliosidosis (GLB1). In the remaining patients, no candidate structural variants, copy number variants or predicted splice variants affecting an extended candidate gene list were identified. Our findings demonstrate the efficacy of using WGS for diagnosing early-onset HSP, particularly in consanguineous families (4/6 diagnosed), highlighting that two of the diagnoses would not have been made using a targeted approach.

Keywords: Gangliosidosis; Hereditary spastic paraplegia; Metabolic; SPG54; SPG56; Whole genome sequencing; Zellweger.

MeSH terms

  • Cytochrome P450 Family 2 / genetics
  • Female
  • Heterozygote
  • Homozygote
  • Humans
  • India
  • Male
  • Membrane Proteins / genetics
  • Mutation
  • Pedigree
  • Phospholipases / genetics
  • Spastic Paraplegia, Hereditary / diagnosis*
  • Spastic Paraplegia, Hereditary / genetics*
  • Whole Genome Sequencing*
  • beta-Galactosidase / genetics

Substances

  • Membrane Proteins
  • PEX16 protein, human
  • CYP2U1 protein, human
  • Cytochrome P450 Family 2
  • Phospholipases
  • DDHD2 protein, human
  • GLB1 protein, human
  • beta-Galactosidase