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A more recent version of this article appeared on April 28, 2000
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Papers In Press, published online ahead of print March 9, 2000
J. Biol. Chem, 10.1074/jbc.C900853199
Submitted on October 18, 1999
Revised on February 28, 2000
Accepted on March 7, 2000

Peripherin/rds influences membrane vesicle morphology: Implications for retinopathies

Jonathan D.J. Wrigley, Tanweer Ahmed, Claire L. Nevett, and John B.C. Findlay

University of Leeds, LS2 9JT, Leeds

Corresponding Author: j.b.c.findlay{at}leeds.ac.uk

Peripherin/rds is an integral membrane glycoprotein found in the rim regions of vertebrate photoreceptor cell discs. Natural mutations of the encoding gene result in degenerative retinal disorders, such as retinitis pigmentosa (R.P.). The ?Retinal degeneration slow? (rds) phenotype, observed in mice, is considered to be an appropriate model for peripherin/rds-mediated R.P. Associated abnormalities in the outer segment of photoreceptor cells have implicated peripherin/rds in some aspect of disc morphology, yet it remains unclear whether such morphological effects are the cause or the result of this condition. Here we present the first direct evidence to support a role for peripherin/rds in maintaining the flattened vesicle morphology characteristic of photoreceptor outer segments. In vitro expression yields a 36 kDa immunoreactive species which is inserted into membranes and undergoes N-glycosylation, inter- and intramolecular disulphide bonding and dimerisation. Electron microscopy reveals that peripherin/rds flattens microsomal vesicles. This effect appears to be dependent on disulphide bond formation but not N-glycosylation. The inability of two pathogenic peripherin/rds mutants (P216L and C165Y) to flatten membrane vesicles implicates such mutations as the primary cause of the retinal degeneration observed in retinitis pigmentosa.


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