Lysyl hydroxylase 3 is required for normal lens capsule formation and maintenance of lens epithelium integrity and fate

Kineret Taler, Omri Weiss, Shahar Rotem-Bamberger, Ariel M. Rubinstein, Pawat Seritrakul, Jeffrey M. Gross, Adi Inbal*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


Lens abnormalities are a major cause of reduced vision and blindness. One mechanism that can lead to reduced lens transparency, i.e. cataract, is abnormal behavior of lens epithelial cells (LECs), the precursors of the transparent lens fiber cells. Here we describe a zebrafish mutation causing the embryonic lens epithelium to generate cellular masses comprising partially differentiated lens fiber cells. We identify the mutant gene as plod3, which encodes for Lysyl hydroxylase 3 (Lh3), an enzyme essential for modification of collagens, including Collagen IV, a main component of the lens capsule. We show that plod3-deficient lenses have abnormal lens epithelium from an early developmental stage, as well as abnormal lens capsules. Subsequently, upregulation of TGFβ signaling takes place, which drives the formation of lens epithelial cellular masses. We identify a similar phenotype in Collagen IVα5-deficient embryos, suggesting a key role for the defective lens capsule in the pathogenesis. We propose that plod3 and col4a5 mutant zebrafish can serve as useful models for better understanding the biology of LECs during embryonic development and in formation of lens epithelium-derived cataract.

Original languageAmerican English
Pages (from-to)177-188
Number of pages12
JournalDevelopmental Biology
Issue number2
StatePublished - 15 Feb 2020

Bibliographical note

Funding Information:
This research was supported by the Israel Science Foundation ( ISF 801/17 to A.I.) and National Institutes of Health / National Eye Institute ( R21-EY22710 to J.M.G.).

Publisher Copyright:
© 2019 Elsevier Inc.


  • Lens capsule
  • Lens epithelium
  • Lysyl hydroxylase 3
  • TGFβ
  • Zebrafish
  • plod3


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