Mutations in the gene encoding the low-density lipoprotein receptor LRP4 cause abnormal limb development in the mouse

Genomics. 2006 May;87(5):673-7. doi: 10.1016/j.ygeno.2006.01.007. Epub 2006 Mar 6.

Abstract

Positional cloning of two recessive mutations of the mouse that cause polysyndactyly (dan and mdig-Chr 2) confirmed that the gene encoding MEGF7/LRP4, a member of the low-density lipoprotein receptor family, plays an essential role in the process of digit differentiation. Pathologies observed in the mutant mice provide insight into understanding the function(s) of LRP4 as a negative regulator of the Wnt-beta-catenin signaling pathway and may help identify the genetic basis for common human disorders with similar phenotypes.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Animals, Genetically Modified
  • Base Sequence
  • DNA Mutational Analysis
  • Female
  • Forelimb / abnormalities*
  • Forelimb / diagnostic imaging
  • LDL-Receptor Related Proteins
  • Male
  • Mice
  • Molecular Sequence Data
  • Mutagenesis, Insertional
  • Mutation
  • Open Reading Frames
  • Radiography
  • Receptors, LDL / genetics*
  • Reverse Transcriptase Polymerase Chain Reaction / methods
  • Sequence Analysis, DNA
  • Syndactyly / diagnosis
  • Syndactyly / genetics

Substances

  • LDL-Receptor Related Proteins
  • Lrp4 protein, mouse
  • Receptors, LDL

Associated data

  • GENBANK/AF247636
  • GENBANK/AF247637