AIMS Mutations of seven crystallin genes have been shown to cause familial cataract. The authors aimed to identify disease causing crystallin mutations in paediatric cataract families from south eastern Australia. METHODS 38 families with autosomal dominant or recessive paediatric cataract were examined. Three large families were studied by linkage analysis. Candidate genes at regions providi...

We have chemically crosslinked a globular protein, gamma IIIb-crystallin, to produce a system of well-defined oligomers: monomers, dimers, trimers and a mixture of higher n-mers. Gel electrophoresis, size exclusion chromatography, quasielastic light scattering spectroscopy, and electrospray ionization mass spectrometry were used to characterize the oligomers formed. The liquid-liquid phase sepa...

The transparency of the eye lens depends on the high solubility and stability of the lens crystallin proteins. The monomeric gamma-crystallins and oligomeric beta-crystallins have paired homologous double Greek key domains, presumably evolved through gene duplication and fusion. Prior investigation of the refolding of human gammaD-crystallin revealed that the C-terminal domain folds first and n...

PURPOSE To investigate the altered expression of proteins in the lens of mice with inherited cataracts. METHODS Mice with inherited cataracts caused by a spontaneous mutation of the gene gamma S-crystallin (Crygs) were used as the subjects. Lens proteins were extracted and separated by two-dimensional electrophoresis (2-DE). The spots representing differential proteins were first identified b...

Aim: Comparative immunohistochemical study of expression α A Crystallin in non-cataract lenses and age-related cataract humans. Methodology: This was an observational cross sectional study. There are two groups this Group comprised 121 senile degenerative from diagnosed patients. B included 10 patients who underwent surgeries for enucleation due to trauma retinoblastoma. Lenses were fixed 10% B...

A quantitative analysis of cell division and cell elongation was carried out during lens morphogenesis in the rat. At 13 days of development elongating cells in the posterior part of the lens vesicle (presumptive fibre cells) have a lower mitotic activity than cells in the anterior vesicle. By 14 days these elongating cells do not divide. Thus at 14 days of development the lens can be separated...

PURPOSE To measure the protein distribution patterns in single young porcine lenses. METHODS Twenty fresh porcine lenses from 5 to 6 months old animals were fractionated into 8-10 concentric fractions by controlled dissolution in phosphate buffer. Proportions of soluble and insoluble protein were determined by Bradford assay. Water-soluble proteins in all layers were separated into HMW, MMW, ...

PURPOSE Mature-onset cataract results from the formation of light-scattering aggregates of lens crystallins. Although oxidative or mutational damage may be a prerequisite, little is known of the initiation or nucleation of these aggregated states. In mice carrying mutations in gamma-crystallin genes, a truncated form of gamma-crystallin formed intranuclear filamentous inclusions within lens fib...

Timolol is a non-selective beta-adrenergic receptor antagonist administered for treating glaucoma, heart attacks and hypertension. In the present study, we set out to determine whether or not timolol can provoke cataract formation, thus the influence of timolol on the amyloid-type aggregation of crystallin was investigated. We then provided experimental evidence of crystallin aggregation and it...