Journal
JOURNAL OF STRUCTURAL BIOLOGY
Volume 129, Issue 1, Pages 38-47Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1006/jsbi.1999.4202
Keywords
3D reconstruction; full thickness macular hole; age-related macular degeneration; eye vitreous; network forming collagens
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Transversely banded deposits with an approximately 100-nm periodicity have been seen in association with a number of eye pathologies (e.g., age-related macular degeneration). Recently such aggregates have also been discovered in the cortical vitreous of a patient suffering from full thickness macular holes. The aggregates in the vitreous were of sufficient size and regularity for us to attempt 3D ultrastructural studies in the electron microscope. The molecules forming this aggregate pack in a centered tetragonal unit cell of dimensions similar to 26 x 26 x 180 nm. A real-space (r-weighted back projection) 3D reconstruction was computed. The aggregate is discussed in terms of its possible protein constituents. Collagen VI has been singled out as the most likely protein to form the aggregate. Two alternative models for the molecular packing are proposed, comprising aggregates of molecular tetramers or octamers, Understanding the structure of these abnormal banded deposits in the eye should help to throw light on the pathophysiological mechanisms of the diseases, including age-related macular degeneration, in which they occur. (C) 2000 Academic Press.
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