Anterior lens epithelium in cataract patients with retinitis pigmentosa – scanning and transmission electron microscopy study

Andjelić, Sofija; Drašlar, Kazimir; Hvala, Anastazija; Hawlina, Marko

doi:10.1111/aos.13250

Cited by 20 publications

(11 citation statements)

References 27 publications

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“…Informed consents were obtained from all donors and their family members. Human ALCs were obtained by a single ophthalmologist from anterior surface of cataractous lenses during surgery, using the central circular capsulorhexis method [101]. Following careful dissection from cataractous lenses, ALCs were temporarily stored in PBS at 4°C.…”

Section: Methodsmentioning

confidence: 99%

Laminin α4 overexpression in the anterior lens capsule may contribute to the senescence of human lens epithelial cells in age-related cataract

Sun

et al. 2019

Aging

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Senescence is a leading cause of age-related cataract (ARC). The current study indicated that the senescence-associated protein, p53, total laminin (LM), LMα4, and transforming growth factor-beta1 (TGF-β1) in the cataractous anterior lens capsules (ALCs) increase with the grades of ARC. In cataractous ALCs, patient age, total LM, LMα4, TGF-β1, were all positively correlated with p53. In lens epithelial cell (HLE B-3) senescence models, matrix metalloproteinase-9 (MMP-9) alleviated senescence by decreasing the expression of total LM and LMα4; TGF-β1 induced senescence by increasing the expression of total LM and LMα4. Furthermore, MMP-9 silencing increased p-p38 and LMα4 expression; anti-LMα4 globular domain antibody alleviated senescence by decreasing the expression of p-p38 and LMα4; pharmacological inhibition of p38 MAPK signaling alleviated senescence by decreasing the expression of LMα4. Finally, in cataractous ALCs, positive correlations were found between LMα4 and total LM, as well as between LMα4 and TGF-β1. Taken together, our results implied that the elevated LMα4, which was possibly caused by the decreased MMP-9, increased TGF-β1 and activated p38 MAPK signaling during senescence, leading to the development of ARC. LMα4 and its regulatory factors show potential as targets for drug development for prevention and treatment of ARC.

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Section: Methodsmentioning

confidence: 99%

Laminin α4 overexpression in the anterior lens capsule may contribute to the senescence of human lens epithelial cells in age-related cataract

Sun

et al. 2019

Aging

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“…It has to be particularly noted that the oxidative mechanism for the formation of nuclear cataracts differs from that of cortical cataracts: the paper from Neale et al suggests that the majority of UV-induced lens damage occurs before 30 years of age in the cortical lens fibres progressively pushed to the centre of the nucleus with the ageing of the lens, supporting the role of a cumulative effect of occupational solar radiation exposure, especially if started at early ages. Finally, the data on PCS are less conclusive: this form is typical, for example, of subjects with a history of chronic use of steroid drugs or with specific ocular comorbidities (Andjelic et al 2017), while no clear association with outdoor work emerges.…”

Section: Associations Between Cataract Its Main Subtypes and Occupatmentioning

confidence: 96%

Cataract frequency and subtypes involved in workers assessed for their solar radiation exposure: a systematic review

Modenese

Gobba

2018

Acta Ophthalmologica

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Cataract is currently the primary cause of blindness worldwide, and one of its main risk factors is solar ultraviolet radiation exposure. According to the localization of lens opacities, three main subtypes of cataract are recognized: nuclear, cortical and posterior subcapsular cataract. One of the main determinants of individual long-term solar radiation exposure is outdoor work. We systematically reviewed scientific literature from the last 20 years to update the recent development of research on the risk of cataract in outdoor workers and on the specific subtypes involved, also investigating the methods applied to evaluate the occupational risk. A total of 15 studies were included in the review, of which 12 showed a positive association. The studies confirm the relationship of long-term occupational solar radiation exposure with cortical cataract and give new support for nuclear cataract, although no substantial new data were available to support a relation with the posterior subcapsular subtype. In most of the studies, the exposure assessment was not adequate to support a representative evaluation of the ocular risk; however, outdoor work is clearly a relevant risk factor for cataract. Further research providing a better evaluation of the relation between solar radiation exposure levels and lens damage in workers is needed and aimed to establish adequate occupational exposure limits and better preventive measures, studying also their effectiveness.

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“…A recent study examined the lenses in patients with RP following cataract surgery. Scanning and transmission electron microscopy revealed changes in the anterior lens epithelium such as holes, thinning, and degradation, which may cause water influx and thus lens opacification [28]. PDE6Β is present throughout the lens, especially in the lens epithelium of mice where it is thought to regulate cGMP levels to prevent embrittlement of the lens.…”

Section: Discussionmentioning

confidence: 99%

Novel PDE6B Mutation Presenting with Retinitis Pigmentosa – A Case Series of Three Patients

Palmowski-Wolfe

Štingl

Habibi

et al. 2019

Klin Monatsbl Augenheilkd

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Background Retinitis pigmentosa (RP) affects 2.5 million people worldwide. Increased identification of causative gene defects and the increasing possibility of treatment necessitates better knowledge of phenotype-genotype correlations to help identify patients who would benefit from targeted gene therapy and improve patientsʼ care. Here, we report on three RP patients with mutations in the PDE6Β Gene that have not been described previously. History and Signs Three patients with a PDE6Β mutation were identified: 1. A 30-year-old male with a homozygotous mutation (c.[2351dupA],[2351dupA], p.[Q785Gfs*20],[Q785Gfs*20]) who was followed for 8 years. 2. A 54-year-old Caucasian woman with a heterozygous mutation [p.(K611Nfs*6), p.(Q567*)] who was followed for 40 years. 3. A 46-year-old Caucasian male [p.(E271K), p.(R627_E631del)]. All had noted an onset in childhood and complained of night blindness and photophobia. Typical bone spiculae were seen, and peripheral visual fields were progressively affected in all patients. Ganzfeld-ERG showed typical signs of rod-cone dystrophy. Patients 1 and 2 underwent cataract surgery at ages 27 and 36 years with an improvement in vision, while patient 3 had not developed a cataract at age 54. Conclusions In children complaining of night blindness, a PDE6Β-associated RP needs to be taken into consideration. Apart from helping patients with optical aids, such as polarizing filters or magnification, a specific diagnosis is especially important in view of emerging genetic treatment options. In particular, in RP patients with a PDE6Β mutation, a phase I/II study is currently ongoing (https://clinicaltrials.gov/ct2/show/NCT03328130).

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Anterior lens epithelium in cataract patients with retinitis pigmentosa – scanning and transmission electron microscopy study

Cited by 20 publications

References 27 publications

Laminin α4 overexpression in the anterior lens capsule may contribute to the senescence of human lens epithelial cells in age-related cataract

Laminin α4 overexpression in the anterior lens capsule may contribute to the senescence of human lens epithelial cells in age-related cataract

Cataract frequency and subtypes involved in workers assessed for their solar radiation exposure: a systematic review

Novel PDE6B Mutation Presenting with Retinitis Pigmentosa – A Case Series of Three Patients

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