Necrotic enlargement of cone photoreceptor cells and the release of high-mobility group box-1 in retinitis pigmentosa

Y Murakami; Y Ikeda; S Nakatake; T Tachibana; K Fujiwara; N Yoshida; S Notomi; S Nakao; T Hisatomi; J W Miller; DG Vavvas; KH Sonoda; T Ishibashi

doi:10.1038/cddiscovery.2015.58

Necrotic enlargement of cone photoreceptor cells and the release of high-mobility group box-1 in retinitis pigmentosa

Cell Death Discovery ◽

10.1038/cddiscovery.2015.58 ◽

2015 ◽

Vol 1 (1) ◽

Cited By ~ 9

Author(s):

Y Murakami ◽

Y Ikeda ◽

S Nakatake ◽

T Tachibana ◽

K Fujiwara ◽

...

Keyword(s):

Retinitis Pigmentosa ◽

High Mobility ◽

Photoreceptor Cells ◽

High Mobility Group ◽

Cone Photoreceptor

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Cone photoreceptor dysfunction in retinitis pigmentosa revealed by optoretinography

Proceedings of the National Academy of Sciences ◽

10.1073/pnas.2107444118 ◽

2021 ◽

Vol 118 (47) ◽

pp. e2107444118

Author(s):

Ayoub Lassoued ◽

Furu Zhang ◽

Kazuhiro Kurokawa ◽

Yan Liu ◽

Marcel T. Bernucci ◽

...

Keyword(s):

Retinitis Pigmentosa ◽

Adaptive Optics ◽

Vision Loss ◽

Photoreceptor Cells ◽

Segment Length ◽

Cone Photoreceptor ◽

Cone Cells ◽

Common Group ◽

Cone Density

Retinitis pigmentosa (RP) is the most common group of inherited retinal degenerative diseases, whose most debilitating phase is cone photoreceptor death. Perimetric and electroretinographic methods are the gold standards for diagnosing and monitoring RP and assessing cone function. However, these methods lack the spatial resolution and sensitivity to assess disease progression at the level of individual photoreceptor cells, where the disease originates and whose degradation causes vision loss. High-resolution retinal imaging methods permit visualization of human cone cells in vivo but have only recently achieved sufficient sensitivity to observe their function as manifested in the cone optoretinogram. By imaging with phase-sensitive adaptive optics optical coherence tomography, we identify a biomarker in the cone optoretinogram that characterizes individual cone dysfunction by stimulating cone cells with flashes of light and measuring nanometer-scale changes in their outer segments. We find that cone optoretinographic responses decrease with increasing RP severity and that even in areas where cone density appears normal, cones can respond differently than those in controls. Unexpectedly, in the most severely diseased patches examined, we find isolated cones that respond normally. Short-wavelength–sensitive cones are found to be more vulnerable to RP than medium- and long-wavelength–sensitive cones. We find that decreases in cone response and cone outer-segment length arise earlier in RP than changes in cone density but that decreases in response and length are not necessarily correlated within single cones.

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