Human disease phenotypes associated with mutations in TREX1

J Clin Immunol. 2015 Apr;35(3):235-43. doi: 10.1007/s10875-015-0147-3. Epub 2015 Mar 4.

Abstract

Considering that it is a single exon gene encoding a 314 amino acid protein, the genotype-phenotype landscape of TREX1 is remarkably complex. Here we briefly describe the human diseases so-far associated with mutations in TREX1, which include Aicardi-Goutières syndrome, familial chilblain lupus, systemic lupus erythematosus and retinal vasculopathy with cerebral leukodystrophy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Autoimmune Diseases of the Nervous System / genetics*
  • Chilblains / genetics*
  • Exodeoxyribonucleases / genetics*
  • Hereditary Central Nervous System Demyelinating Diseases / genetics*
  • Humans
  • Lupus Erythematosus, Cutaneous / genetics*
  • Lupus Erythematosus, Systemic / genetics*
  • Mutation
  • Nervous System Malformations / genetics*
  • Phenotype
  • Phosphoproteins / genetics*
  • Retinal Diseases / genetics*
  • Vascular Diseases / genetics*

Substances

  • Phosphoproteins
  • Exodeoxyribonucleases
  • three prime repair exonuclease 1

Supplementary concepts

  • Aicardi-Goutieres syndrome
  • Chilblain lupus 1
  • Vasculopathy, Retinal, With Cerebral Leukodystrophy