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Biochemical and biomechanical characteristics of dystrophin-deficient mdx3cv mouse lens.

Publication ,  Journal Article
Karnam, S; Skiba, NP; Rao, PV
Published in: Biochim Biophys Acta Mol Basis Dis
January 1, 2021

The molecular and cellular basis for cataract development in mice lacking dystrophin, a scaffolding protein that links the cytoskeleton to the extracellular matrix, is poorly understood. In this study, we characterized lenses derived from the dystrophin-deficient mdx3cv mouse model. Expression of Dp71, a predominant isoform of dystrophin in the lens, was induced during lens fiber cell differentiation. Dp71 was found to co-distribute with dystroglycan, connexin-50 and 46, aquaporin-0, and NrCAM as a large cluster at the center of long arms of the hexagonal fibers. Although mdx3cv mouse lenses exhibited dramatically reduced levels of Dp71, only older lenses revealed punctate nuclear opacities compared to littermate wild type (WT) lenses. The levels of dystroglycan, syntrophin, and dystrobrevin which comprise the dystrophin-associated protein complex (DAPC), and NrCAM, connexin-50, and aquaporin-0, were significantly lower in the lens membrane fraction of adult mdx3cv mice compared to WT mice. Additionally, decreases were observed in myosin light chain phosphorylation and lens stiffness together with a significant elevation in the levels of utrophin, a functional homolog of dystrophin in mdx3cv mouse lenses compared to WT lenses. The levels of perlecan and laminin (ligands of α-dystroglycan) remained normal in dystrophin-deficient lens fibers. Taken together, although mdx3cv mouse lenses exhibit only minor defects in lens clarity possibly due to a compensatory increase in utrophin, the noted disruptions of DAPC, stability, and organization of membrane integral proteins of fibers, and stiffness of mdx3cv lenses reveal the importance of dystrophin and DAPC in maintaining lens clarity and function.

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Published In

Biochim Biophys Acta Mol Basis Dis

DOI

EISSN

1879-260X

Publication Date

January 1, 2021

Volume

1867

Issue

1

Start / End Page

165998

Location

Netherlands

Related Subject Headings

  • Mice, Inbred mdx
  • Mice
  • Lens, Crystalline
  • Gene Expression Regulation
  • Eye Proteins
  • Dystrophin
  • Biochemistry & Molecular Biology
  • Animals
  • 3205 Medical biochemistry and metabolomics
  • 3101 Biochemistry and cell biology
 

Citation

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Karnam, S., Skiba, N. P., & Rao, P. V. (2021). Biochemical and biomechanical characteristics of dystrophin-deficient mdx3cv mouse lens. Biochim Biophys Acta Mol Basis Dis, 1867(1), 165998. https://doi.org/10.1016/j.bbadis.2020.165998
Karnam, Shruthi, Nikolai P. Skiba, and Ponugoti V. Rao. “Biochemical and biomechanical characteristics of dystrophin-deficient mdx3cv mouse lens.Biochim Biophys Acta Mol Basis Dis 1867, no. 1 (January 1, 2021): 165998. https://doi.org/10.1016/j.bbadis.2020.165998.
Karnam S, Skiba NP, Rao PV. Biochemical and biomechanical characteristics of dystrophin-deficient mdx3cv mouse lens. Biochim Biophys Acta Mol Basis Dis. 2021 Jan 1;1867(1):165998.
Karnam, Shruthi, et al. “Biochemical and biomechanical characteristics of dystrophin-deficient mdx3cv mouse lens.Biochim Biophys Acta Mol Basis Dis, vol. 1867, no. 1, Jan. 2021, p. 165998. Pubmed, doi:10.1016/j.bbadis.2020.165998.
Karnam S, Skiba NP, Rao PV. Biochemical and biomechanical characteristics of dystrophin-deficient mdx3cv mouse lens. Biochim Biophys Acta Mol Basis Dis. 2021 Jan 1;1867(1):165998.

Published In

Biochim Biophys Acta Mol Basis Dis

DOI

EISSN

1879-260X

Publication Date

January 1, 2021

Volume

1867

Issue

1

Start / End Page

165998

Location

Netherlands

Related Subject Headings

  • Mice, Inbred mdx
  • Mice
  • Lens, Crystalline
  • Gene Expression Regulation
  • Eye Proteins
  • Dystrophin
  • Biochemistry & Molecular Biology
  • Animals
  • 3205 Medical biochemistry and metabolomics
  • 3101 Biochemistry and cell biology