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1 ees of visual dysfunction in mouse models of retinal degeneration.
2 lishes critical vulnerability for later aged retinal degeneration.
3 reby restoring vision in patients blinded by retinal degeneration.
4 linical trial for the treatment of inherited retinal degeneration.
5 tion between pesticide use and self-reported retinal degeneration.
6 the underlying cause of recessive inherited retinal degeneration.
7 nization of RPE cells, ultimately leading to retinal degeneration.
8 rt demonstrating the involvement of IFT43 in retinal degeneration.
9 nifested by damage to the nervous system and retinal degeneration.
10 ts explain the likely cause of PD-associated retinal degeneration.
11 clinically beneficial for patients with this retinal degeneration.
12 re associated with a spectrum of progressive retinal degeneration.
13 t exposure, and Fam161a-associated inherited retinal degeneration.
14 tic features of CLN3-associated nonsyndromic retinal degeneration.
15 P6 in mice (Reep6-/-) results in progressive retinal degeneration.
16 or vision restoration in patients blinded by retinal degeneration.
17 in the retina is a therapeutic strategy for retinal degeneration.
18 rocess in the P23H-1 transgenic rat model of retinal degeneration.
19 as a therapeutic agent for treating ischemic retinal degeneration.
20 on of foveal vision in eyes with MAK-related retinal degeneration.
21 neuronal cell death, including in hereditary retinal degeneration.
22 in the retina is a therapeutic strategy for retinal degeneration.
23 apeutic retinoid that prevents light-induced retinal degeneration.
24 is neuroprotective against pressure mediated retinal degeneration.
25 he first 6 wk of life, as a model for severe retinal degeneration.
26 tinal protein and potent cause of autoimmune retinal degeneration.
27 d BBS-related phenotypes as well as isolated retinal degeneration.
28 result of retinitis pigmentosa (RP) or outer retinal degeneration.
29 lines developed progressive and severe outer retinal degeneration.
30 y have potential as a strategy for reversing retinal degeneration.
31 while MFRP families were more likely to have retinal degeneration.
32 ch into the role that these pathways play in retinal degeneration.
33 ial future treatment for blindness caused by retinal degeneration.
34 aturally occurring chicken mutation leads to retinal degeneration.
35 on products in Mertk(-/-) mouse RPE prior to retinal degeneration.
36 therapeutic strategies at earlier stages of retinal degeneration.
37 typic variability in mutant PRPH2-associated retinal degeneration.
38 ifferences, including distinct mechanisms of retinal degeneration.
39 t a retinoid-independent mechanism underlies retinal degeneration.
40 functional in the retina and thus prevented retinal degeneration.
41 igmentosa (XLRP) and 15-20% of all inherited retinal degeneration.
42 n many neurodegenerative diseases, including retinal degeneration.
43 ity and some vision to mice blind from outer retinal degeneration.
44 ons for patients with debilitating inherited retinal degeneration.
45 urred prior to structural changes of RPE and retinal degeneration.
46 f this gene results in a severe, early-onset retinal degeneration.
47 tion triggers a dominant form of progressive retinal degeneration.
48 restoration of sight in patients blinded by retinal degeneration.
49 ease severity in patients with USH2A-related retinal degeneration.
50 visual function in a mouse model of advanced retinal degeneration.
51 is gene can also lead to an isolated form of retinal degeneration.
52 es, and apoptosis, that are activated during retinal degeneration.
53 lammation, vitreous and retinal fibrosis and retinal degeneration.
54 wild-type C57BL/6J mice without discernible retinal degeneration.
55 ne are a common cause of autosomal recessive retinal degeneration.
56 d-type (WT) activity, causes relatively mild retinal degeneration.
57 r atRAL via Schiff base formation ameliorate retinal degeneration.
58 SPP2 has a new role in retinal degeneration.
59 s the first strain of mice identified with a retinal degeneration.
60 actuator for treating patients with advanced retinal degeneration.
61 play an important role in protecting against retinal degeneration.
62 tivity and RGC morphology at early stages of retinal degeneration.
63 e to NPHP with cerebellar vermis aplasia and retinal degeneration.
64 capitulated the human phenotypes of NPHP and retinal degeneration.
65 e neurodevelopmental abnormalities and neuro-retinal degeneration.
66 hereditary genetic disease causing bilateral retinal degeneration.
67 gos28 lead to defective Rh1 trafficking and retinal degeneration.
68 re visual function in patients with regional retinal degeneration.
69 e early stage of STGD1 since it is devoid of retinal degeneration.
70 constitutive activity and the potential for retinal degeneration.
71 000 lx of light for thirty minutes to induce retinal degeneration.
72 Fam151b homozygous knock-out mice as having retinal degeneration.
73 nal stressors implicated in the mechanism of retinal degeneration.
74 RALBP), cause an autosomal recessive form of retinal degeneration.
75 hereas homozygous mice exhibited progressive retinal degeneration.
76 , which is generally thought to be devoid of retinal degeneration.
77 iving pigmented or albino rats and mice with retinal degeneration.
78 0) gene cause various ciliopathies involving retinal degeneration.
79 nsensitive retinal cones in a mouse model of retinal degeneration.
80 chondrial number and size prior any signs of retinal degeneration.
81 86fs), and presented with severe early-onset retinal degeneration.
82 nts in SSBP1 as a cause of ADOA and variable retinal degeneration.
83 vision in RP and potentially other forms of retinal degeneration.
84 a sham procedure, slowed the progression of retinal degeneration.
85 lies and 2 singletons with ADOA and variable retinal degeneration.
86 n-related anxiety in patients with inherited retinal degenerations.
87 indow for successful gene therapy in certain retinal degenerations.
88 singly found to cause nonsyndromic inherited retinal degenerations.
89 and reveal potential therapeutic targets for retinal degenerations.
90 fied in patients with nonsyndromic inherited retinal degenerations.
91 ealed clues to the causes of the progressive retinal degenerations.
92 in the evaluation of patients with inherited retinal degenerations.
93 ne-specific therapeutic avenue for inherited retinal degenerations.
94 or structural defects to trigger progressive retinal degenerations.
95 monitoring tool for patients with inherited retinal degenerations.
97 of the common models used to study RP is the retinal degeneration-10 (rd10) mouse, which has a mutati
98 homolog of the human retinal dystrophy gene Retinal Degeneration 3 (RD3) is a Golgi-associated prote
101 the G86R GCAP1-RetGC1 complex inhibition by retinal degeneration 3 (RD3) protein was shifted toward
108 1 in mature postmitotic rods leads to robust retinal degeneration accompanied by loss of visual funct
110 ion in patients who are blind from end-stage retinal degenerations aim to render remaining retinal ce
112 s renal and hepatic cysts, skeletal defects, retinal degeneration and central nervous system malforma
114 ific deletion of MPC1 results in progressive retinal degeneration and decline of visual function in b
116 relatively early onset (by 3 months of age) retinal degeneration and dysfunction when compared with
118 tained from 35 adult patients with inherited retinal degeneration and fibroblast lines were establish
120 c.100 G > A change in IFT43 segregating with retinal degeneration and not present in ethnicity-matche
121 667161 decreases the obesity, liver disease, retinal degeneration and olfaction defect in Bbs2-/- mic
122 a rare neurodegenerative disease with early retinal degeneration and progressive neurologic deterior
123 organelles may contribute to KCNJ13-related retinal degeneration and provide a therapeutic target.
124 i-organ dysfunction, such as cardiomyopathy, retinal degeneration and renal dysfunction, the disorder
125 a platform to investigate the mechanisms of retinal degeneration and screen for neuroprotective comp
126 resulting in mitochondrial dysfunction, and retinal degeneration and that therapies normalizing mito
127 d with early onset non-syndromic progressive retinal degeneration and the presence of bone spicules d
128 (Ca(2+)) in rod photoreceptors are linked to retinal degeneration and visual disorders such as retini
130 ll types in the diseasesettings of inherited retinal degenerations and age-related macular degenerati
131 llary light reflexes, phenotypic presence of retinal degeneration, and a non-recordable electroretino
132 cells to identify how CEP290 mutations cause retinal degeneration, and show an antisense approach can
133 association of p.G122R with milder forms of retinal degeneration, and show that while p.G122R had no
135 understand these mild forms of RPE65-related retinal degeneration, and their effect on cone photorece
136 se is used as a pre-clinical animal model of retinal degeneration, and we found it was also hyperopic
140 nlikely to be a primary mechanism underlying retinal degeneration as most LCA-associated NMNAT1 mutan
141 t not C-whirlin in the inner ear, and led to retinal degeneration as well as moderate to severe heari
144 r rat strain (BN-J) presenting a progressive retinal degeneration associated with early retinal telan
145 ng to a new therapeutic target to counteract retinal degeneration associated with lysosomal dysfuncti
146 n-frame deletions frequently caused dominant retinal degeneration associated with rhodopsin biosynthe
147 r genetic findings in nonsyndromic inherited retinal degenerations associated with CLN3 mutations.
148 rovides insights into the pathomechanisms of retinal degenerations associated with compromised ciliar
151 ht patients with a diagnosis of an inherited retinal degeneration at the Kellogg Eye Center (Universi
152 genetic rescue approach to 'optimally' treat retinal degeneration at various disease stages and exami
154 ow a range of clinical phenotypes, including retinal degeneration, brachydactyly, craniofacial abnorm
155 l ganglion cells to light in mouse models of retinal degeneration but do not recapitulate native reti
156 ation restores visual responses in end-stage retinal degeneration, but has also been assessed in non-
157 ative effects in animal models of stroke and retinal degeneration, but the underlying therapeutic mec
158 enetic mutations in the PROM1 gene result in retinal degeneration by impairing the proper formation o
161 The results of this study demonstrate that retinal degeneration can be stopped, even at late stages
163 presented with rapidly progressive childhood retinal degeneration, cardiomyopathy and almost undetect
166 to amino acid deprivation, susceptibility to retinal degeneration caused by endoplasmic reticulum (ER
168 ients with mutations in PRPH2 exhibit severe retinal degeneration characterized by vast inter- and in
169 sing RBP4 (RBP4-Tg mice) develop progressive retinal degeneration, characterized by photoreceptor rib
170 rsened rod and cone function and exacerbated retinal degeneration compared with Prph2R172W animals.
171 amaurosis 9 (LCA9) is an autosomal recessive retinal degeneration condition caused by mutations in th
172 ing visual function using a rat model of the retinal degeneration condition retinitis pigmentosa.
174 peptides and PEDF in the rd1 mouse model of retinal degeneration decreased the numbers of dying phot
175 eases including glaucoma, optic atrophy, and retinal degeneration--defects in mitochondrial function
176 paired visual responses resulting from outer retinal degeneration diseases such as retinitis pigmento
177 t the hypothesis that RPGR mutations lead to retinal degeneration due to a dysregulation of the actin
178 has been tested in clinical trials for human retinal degeneration due to its potent neuroprotective e
180 In humans, CERKL mutations cause widespread retinal degeneration: early dysfunction and loss of rod
182 thological hyperactivity in a mouse model of retinal degeneration elevates rather than reduces motili
185 bly, we discover that the major isoform of a retinal degeneration gene, CRB1, was previously overlook
186 ven though the pathobiology of the resulting retinal degeneration has been characterized in several a
187 progressive multi-organ pathology including retinal degeneration, hearing impairment and type 2 diab
188 therapy shows promise for treating inherited retinal degenerations; however, relevant animal models a
191 arly-onset, autosomal-recessive, progressive retinal degeneration in Bengal cats; we identified a c.1
193 290 in photoreceptors and pathomechanisms of retinal degeneration in CEP290-associated ciliopathies a
194 is part of the disease mechanisms that cause retinal degeneration in CEP290-associated ciliopathies.
196 rescues both the Rh1 trafficking defects and retinal degeneration in Drosophila gos28 mutants, demons
198 genes that disrupt cGMP homeostasis leads to retinal degeneration in humans through mechanisms that a
205 o find common biological pathways that cause retinal degeneration in various forms of RP, and identif
206 cause rhodopsin mislocalisation and eventual retinal degeneration in XLRP.Mutations in the Retinitis
207 retinal atrophies are a group of hereditary retinal degenerations in dogs characterised by depletion
208 tinal degeneration slow/RDS, lead to various retinal degenerations including retinitis pigmentosa (RP
209 n childhood blindness in seven families with retinal degeneration, including Leber congenital amauros
210 /-)Rdh8(-/-) mice that display light-induced retinal degeneration indicates that 11-cis-retinal and d
211 ysis suggests that VEGF antagonism activates retinal degeneration, inflammation, and other adverse ef
219 roglia activation in the retina suggest that retinal degeneration is driven by a proinflammatory mech
223 itis pigmentosa (RP) is a group of inherited retinal degenerations leading to blindness due to photor
224 oped countries, and is characterized by slow retinal degeneration linked to chronic reactive oxygen s
230 as observed in the widely used light-induced retinal degeneration model and corroborated in other mod
236 (wild-type larvae and adult control animals, retinal degeneration mutants, and light-induced photorec
237 A) with a clinical diagnosis of an inherited retinal degeneration (n = 128) participated in an interv
238 is a rare genetic condition characterised by retinal degeneration, obesity, kidney failure, and cogni
239 and is highly correlated with the extent of retinal degeneration observed in OCT or fundus photograp
242 ted genetically, indicating that some of the retinal degeneration occurred in a transducin-independen
243 nt, phototoxic damage may then cause central retinal degeneration of the vulnerable macula, marked by
244 with activated Wnt functionally rescued the retinal degeneration phenotype in rd10 mice, a model for
245 t NMNAT1 function and ultimately lead to the retinal degeneration phenotype, we performed detailed an
247 formed for pediatric patients with suspected retinal degeneration presenting to a single examiner fro
249 merous ongoing clinical trials for inherited retinal degenerations, quantifiable and reliable outcome
250 nal PRO questionnaire, known as the Michigan Retinal Degeneration Questionnaire, is psychometrically
252 Retinitis pigmentosa (RP) is an inherited retinal degeneration (RD) that leads to blindness for wh
254 variants have been associated with inherited retinal degenerations (RDs) including canine and murine
256 he underlying cellular mechanisms leading to retinal degeneration remain uncertain, although previous
258 nd efficacy trials studying individuals with retinal degeneration resulting from RPE65 mutations-init
259 eclines rapidly in parallel with progressive retinal degeneration, resulting in profound chorioretina
260 ANCE STATEMENT Loss of photoreceptors during retinal degeneration results in permanent visual impairm
261 o those seen in human MD patients, including retinal degeneration, retinal pigment epithlium (RPE) de
262 tral macular lesion of a patient with severe retinal degeneration showed extreme thinning, some prese
264 The photoreceptor-specific glycoprotein retinal degeneration slow (RDS, also called PRPH2) is ne
265 fic gene peripherin-2 (PRPH-2, also known as retinal degeneration slow/RDS) cause incurable retinal d
266 tions in peripherin 2 (PRPH2), also known as retinal degeneration slow/RDS, lead to various retinal d
269 itis pigmentosa (RP) is a group of inherited retinal degenerations that lead to progressive vision lo
270 and Retinitis Pigmentosa (RP) are inherited retinal degenerations that may be affected, in opposite
272 anifestations, including reduced vision with retinal degeneration, the underlying mechanism of which
274 ing transplantation into mice with end-stage retinal degeneration, these cells differentiated into ph
277 neration pedigree with early-onset recessive retinal degeneration to identify the causative mutation.
278 ive families with nephronophthisis (NPH) and retinal degeneration, two of the most common manifestati
280 nopsin gene (OPN4) in the rd1 mouse model of retinal degeneration using an adeno-associated viral vec
281 Scale (WCBS) and the age association of the retinal degeneration using central subfield thickness (C
282 describe the characteristics of MAK-related retinal degeneration using optical coherence tomography
283 al and morphological features of MNU-induced retinal degeneration using scotopic electroretinography
284 fected siblings, and in the 6-years-old, the retinal degeneration was arrested, and the vision was cl
288 orizontal cell phenotype is a consequence of retinal degeneration, we examined this phenotype in mice
290 h are hallmarks of neurological diseases and retinal degenerations, we tested whether hUTCs contribut
292 s mice for the G90D mutation did not exhibit retinal degeneration whereas homozygous mice exhibited p
293 formation and decreased oxidative stress and retinal degeneration, which resulted in improved visual
294 assays indicated the presence of progressive retinal degeneration with a cone predominately affected
295 tinal degeneration slow/RDS) cause incurable retinal degeneration with a high degree of phenotypic va
296 le cystic kidney phenotype along with severe retinal degeneration with mislocalization of phototransd
297 increase in survival, prevented the onset of retinal degeneration with reduced oxidative stress and a
298 Congenital Zika syndrome showed a central retinal degeneration with severe GCL loss, borderline in
299 es FTLD-related behavioral abnormalities and retinal degeneration without improving lipofuscin, C1q,
300 sion, and contribute to visual impairment in retinal degenerations, yet neither the extended network