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Crystallin gamma B-I4F mutant protein binds to alpha-crystallin and affects lens transparency

Academic Article
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Overview

authors

  • Liu, H. Q.
  • Du, X.
  • Wang, M.
  • Huang, Q. L.
  • Ding, L. L.
  • McDonald, H. W.
  • Yates III, John
  • Beutler, Bruce
  • Horwitz, J.
  • Gong, Xiaohua

publication date

  • 2005

journal

  • Journal of Biological Chemistry  Journal

abstract

  • A new mouse mutant line, Clapper, identified from N-ethyl-N-nitrosurea (ENU)-mutagenized mice, develops a dominant lamellar cataract. The cataract blocks the image of retinal fundus and transmits a fuzzy fluorescein image of retinal vasculature during angiography. The cataractous lens opacity decreases as the mice age. The Clapper mutation has been identified to be a missense mutation of the gammaB-crystallin gene that replaces the 4th isoleucine residue with a phenylalanine (gammaB-I4F). Unlike wild type gammaB, the gammaB-I4F mutant protein binds to alpha-crystallin to form high molecular weight complexes in vivo and in vitro. Circular dichroism measurements indicate that gammaB-I4F protein is less stable than wild type gammaB at high temperature. Darkly stained aggregates, enlarged interfiber spaces, and disorganized and smaller inner mature fibers were found in the regions of the cataract in homozygous Clapper mutant lenses. Thus, the lamellar cataract is likely due to the light-scattering effects of the enlarged interfiber spaces and protein aggregates caused by gammaB-I4F mutant proteins interacting with alpha-crystallin in the lens.

subject areas

  • Angiography
  • Animals
  • Chromatography, Gel
  • Circular Dichroism
  • Cloning, Molecular
  • Crystallins
  • DNA Primers
  • Disease Models, Animal
  • Electrophoresis, Gel, Two-Dimensional
  • Ethylnitrosourea
  • Fluorescein
  • Genetic Linkage
  • Homozygote
  • Immunohistochemistry
  • Isoleucine
  • Lens, Crystalline
  • Mice
  • Mice, Mutant Strains
  • Mutagenesis
  • Mutagens
  • Mutation
  • Mutation, Missense
  • Phenotype
  • Phenylalanine
  • Protein Binding
  • Recombinant Proteins
  • Retinal Vessels
  • Sequence Analysis, DNA
  • Ultraviolet Rays
  • alpha-Crystallins
  • gamma-Crystallins
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Identity

International Standard Serial Number (ISSN)

  • 0021-9258

Digital Object Identifier (DOI)

  • 10.1074/jbc.M502490200

PubMed ID

  • 15878859
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Additional Document Info

start page

  • 25071

end page

  • 25078

volume

  • 280

issue

  • 26

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