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2016 ; 150
(ä): 149-65
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Retinal remodeling in human retinitis pigmentosa
#MMPMID27020758
Jones BW
; Pfeiffer RL
; Ferrell WD
; Watt CB
; Marmor M
; Marc RE
Exp Eye Res
2016[Sep]; 150
(ä): 149-65
PMID27020758
show ga
Retinitis Pigmentosa (RP) in the human is a progressive, currently irreversible
neural degenerative disease usually caused by gene defects that disrupt the
function or architecture of the photoreceptors. While RP can initially be a
disease of photoreceptors, there is increasing evidence that the inner retina
becomes progressively disorganized as the outer retina degenerates. These
alterations have been extensively described in animal models, but remodeling in
humans has not been as well characterized. This study, using computational
molecular phenotyping (CMP) seeks to advance our understanding of the retinal
remodeling process in humans. We describe cone mediated preservation of overall
topology, retinal reprogramming in the earliest stages of the disease in retinal
bipolar cells, and alterations in both small molecule and protein signatures of
neurons and glia. Furthermore, while Müller glia appear to be some of the last
cells left in the degenerate retina, they are also one of the first cell classes
in the neural retina to respond to stress which may reveal mechanisms related to
remodeling and cell death in other retinal cell classes. Also fundamentally
important is the finding that retinal network topologies are altered. Our results
suggest interventions that presume substantial preservation of the neural retina
will likely fail in late stages of the disease. Even early intervention offers no
guarantee that the interventions will be immune to progressive remodeling.
Fundamental work in the biology and mechanisms of disease progression are needed
to support vision rescue strategies.
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