Zebrafish as a model for crystallin-associated congenital cataracts in humans [PDF]
Frontiers in Cell and Developmental BiologyCongenital cataracts are a leading cause of vision loss in children and can be an isolated finding or associated with systemic abnormalities. Isolated congenital cataracts are most commonly associated with pathogenic variants in one of the Crystallin ...
Jennifer L. Rossen +5 more
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Characterization of the Interaction of Human γS Crystallin with Metal Ions and Its Effect on Protein Aggregation [PDF]
BiomoleculesCataracts are diseases characterized by the opacity of the ocular lens and the subsequent deterioration of vision. Metal ions are one of the factors that have been reported to induce crystallin aggregation.
Reinier Cardenas +3 more
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The Functional Significance of High Cysteine Content in Eye Lens γ-Crystallins [PDF]
BiomoleculesCataract disease is strongly associated with progressively accumulating oxidative damage to the extremely long-lived crystallin proteins of the lens. Cysteine oxidation affects crystallin folding, interactions, and light-scattering aggregation especially
Eugene Serebryany +2 more
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G91-deletion in βA3/A1-crystallin induces cellular and molecular changes in mouse lenses leading to congenital cataract development. [PDF]
PLoS ONECRYβA1-ΔG91 (βA3ΔG91) is a mutational hotspot in CRYβA1, which causes autosomal dominant congenital nuclear cataract in humans and mice. Previous in-vitro studies of recombinant βA3ΔG91 showed defective folding, decreased solubility, and aberrant ...
Akosua K Boateng +2 more
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Mollusc Crystallins: Physical and Chemical Properties and Phylogenetic Analysis
Diversity, 2022 The purpose of the present study was to perform bioinformatic analysis of crystallin diversity in aquatic molluscs based on the sequences in the NCBI Protein database.
Irina N. Dominova, Valery V. Zhukov
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Zinc and Copper Ions Induce Aggregation of Human β-Crystallins
Molecules, 2022 Cataracts are defined as the clouding of the lens due to the formation of insoluble protein aggregates. Metal ions exposure has been recognized as a risk factor in the cataract formation process. The γ and β crystallins are members of a larger family and
Vanesa Ramirez-Bello +3 more
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Protective Effects of Acetylation on the Pathological Reactions of the Lens Crystallins with Homocysteine Thiolactone. [PDF]
PLoS ONE, 2016 Various post-translational lens crystallins modifications result in structural and functional insults, contributing to the development of lens opacity and cataract disorders.
Zeinab Moafian +4 more
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Analysis of the cytoprotective role of α-crystallins in cell survival and implication of the αA-crystallin C-terminal extension domain in preventing Bax-induced apoptosis. [PDF]
PLoS ONE, 2013 α-Crystallins, initially described as the major structural proteins of the lens, belong to the small heat shock protein family. Apart from their function as chaperones, α-crystallins are involved in the regulation of intracellular apoptotic signals.
Séverine Hamann +3 more
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Frontiers in Pharmacology, 2022 C-kit/CD117, expressed in a series of tissue-specific progenitor cells, plays an important role in tissue regeneration and tissue homeostasis. We previously demonstrated that organoid-derived c-kit+ retinal progenitor cells can facilitate the restoration
Xi Chen +6 more
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Scientific Reports, 2021 Crystallins are ubiquitous, however, prevalence is seen in eye lens. Eye lens crystallins are long-lived and structural intactness is required for maintaining lens transparency and protein solubility. Mutations in crystallins often lead to cataract.
Sunita Patel, Ramakrishna V. Hosur
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