By E16.5, the secondary lens fibre cells were abnormally arranged with poor lens suture formation. Apoptotic cells were found in the centre of the lens as shown by TUNEL assay, the cytoskeleton and cell adhesion in the lens centre were disturbed as shown in immunohistochemistry analysis. By western blotting we found that mutant c-crystallins were reduced in amount and enriched in the insoluble ...

Crystallins which form the major protein components of the eye lens are responsible for its transparency. βγ-crystallin superfamily has a well-characterized protein fold which is present in several members in prokaryotes and eukaryotes. Apart from two members from eukaryote, Spherulin 3a and Ciona crystallin, a majority of them possess the two or more βγ-crystallin domains. The βγ domain consis...

Water soluble proteins (WSPs) in Sus scrofa lenses from pigs in different developmental stages: (young (GI), young adult (GII), and middle-aged (GIII)) were separated using GF-HPLC, yielding fractions of different molecular weights. Non-tryptophan (345/420 nm) and tryptophan (280/345 nm) fluorescence was measured in these fractions. Relative non-tryptophan fluorescence increased with age at a r...

Familial Danish Dementia (FDD) is an autosomal disease, which is distinguished by gradual loss of vision, deafness, progressive ataxia and dementia. Cataract is the first manifestation of the disease. In this article, we demonstrate a specific correlation between the poisoning of the chaperone activity of the rat eye lens alpha-crystallins, loss of lens transparency in organ culture by the path...

The comparison of the chaperone-like activity of native and covalently cross-linked human alphaB-crystallins has confirmed the important role of the subunit mobility in the chaperoning mechanism. Our data clearly demonstrate that the chaperone-like activity of alpha-crystallin is not only a surface phenomenon as was suggested by some researchers.

Adult retinal ganglion cells (RGCs) can regenerate their axons in vitro. Using proteomics, we discovered that the supernatants of cultured retinas contain isoforms of crystallins with crystallin beta b2 (crybb2) being clearly up-regulated in the regenerating retina. Immunohistochemistry revealed the expression of crybb within the retina, including in filopodial protrusions and axons of RGCs. Cl...