Glaucoma Associated with Polycystic Kidney Disease

Berkley, William L.

doi:10.1016/0002-9394(51)90158-4

Cited by 5 publications

(2 citation statements)

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“…For example, the polycystin complex, which is assembled by TRPP2 and its molecular chaperones of polycystin-1, can be activated and induced Ca 2+ influx to primary by mechanical pressure on several stress-bearing cells, like kidney cells [ 8 ], vascular endothelial cells [ 9 ], vascular smooth muscle cells [ 16 ], and bronchial smooth muscle cells [ 17 ]. A case report showed that a polycystic kidney disease (PKD) patient had experienced rapid loss of vision during the terminal stages of his illness, and the postmortem examination revealed glaucomatous changes in both eyes [ 18 ]. Thus, there might be a potential connection between glaucoma and polycystic kidney disease secondary to TRPP2.…”

Section: Discussionmentioning

confidence: 99%

TRPP2 is located in the primary cilia of human non-pigmented ciliary epithelial cells

Zheng

Ziemssen

Suesskind

et al. 2023

Graefes Arch Clin Exp Ophthalmol

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Purpose Mechanosensitive channels (MSCs) and primary cilium possess a possible relevance for the sensation of intraocular pressure (IOP). However, there is only limited data on their expression and localization in the ciliary body epithelium (CBE). The purpose of this study was to characterize the expression and localization of TRPP2 in a human non-pigmented ciliary epithelial cell (HNPCE) line. Methods The expression of the TRPP2 was studied by quantitative (q)RT-PCR and in situ hybridization in rat and human tissue. Protein expression and distribution were studied by western blot analysis, immunohistochemistry, and immunoelectron microscopy. Cellular location of TRPP2 was determined in rat and human CBE by immunofluorescence and immunoblot analysis. Electron microscopy studies were conducted to evaluate where and with substructure TRPP2 is localized in the HNPCE cell line. Results The expression of TRPP2 in rat and human non-pigmented ciliary epithelium was detected. TRPP2 was mainly located in nuclei, but also showed a punctate distribution pattern in the cytoplasm of HNPCE of the tissue and the cell line. In HNPCE cell culture, primary cilia did exhibit different length following serum starvation and hydrostatic pressure. TRPP2 was found to be colocalized with these cilia in HNPCE cells. Conclusion The expression of TRPP2 and the primary cilium in the CB may indicate a possible role, such as the sensing of hydrostatic pressure, for the regulation of IOP. Functional studies via patch clamp or pharmacological intervention have yet to clarify the relevance for the physiological situation or aqueous humor regulation.

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Section: Discussionmentioning

confidence: 99%

TRPP2 is located in the primary cilia of human non-pigmented ciliary epithelial cells

Zheng

Ziemssen

Suesskind

et al. 2023

Graefes Arch Clin Exp Ophthalmol

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“…Intriguingly, there are reports of a tentative association between polycystic kidney disease and retinal defects in patients and experimental models of eye disease (Berkley, 1951, Narendran et al, 2004, Gallagher et al, 2006, Feng et al, 2009). In a rare syndrome, neonates were diagnosed with diabetes, congenital hypothyroidism, glaucoma, hepatic fibrosis and polycystic kidneys (Senee et al, 2006).…”

Section: Discussionmentioning

confidence: 99%

Distribution and function of polycystin-2 in mouse retinal ganglion cells

et al. 2012

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The polycystin family of transient receptor potential (TRP) channels form Ca2+ regulated cation channels with distinct subcellullar localizations and functions. As part of heteromultimeric channels and multi-protein complexes, polycystins control intracellular Ca2+ signals and more generally the translation of extracellular signals and stimuli to intracellular responses. Polycystin-2 channels have been cloned from retina, but their distribution and function in retinal ganglion cells (RGCs) have not yet been established. In the present study, we determined cellular and subcellular localization as well as functional properties of polycystin-2 channels in RGCs. Polycystin-2 expression and distribution in RGCs was assessed by immunohistochemistry on vertical cryostat section of mouse retina as well as primary cultured mouse RGCs, using fluorescence microscopy. Biophysical and pharmacological properties of polycystin-2 channels isolated from primary cultured RGCs were determined using planar lipid bilayer electrophysiology. We detected polycystin-2 immunoreactivity both in the ganglion cell layer as well as in primary cultured RGCs. Subcellular analysis revealed strong cytosolic localization pattern of polycystin-2. Polycystin-2 channel current was Ca2+ activated, had a maximum slope conductance of 114 pS and could be blocked in a dose-dependent manner by increasing concentrations of Mg2+. The cytosolic localization of polycystin-2 in RGCs is in accordance with its function as intracellular Ca2+ release channel. We conclude that polycystin-2 forms functional channels in RGCs, of which biophysical and pharmacological properties are similar to polycystin-2 channels reported for other tissues and organisms. Our data suggest a potential role for polycystin-2 in RGC Ca2+ signaling.

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Klinische Formen des Glaukoms und Hypothesen über die Ursachen

Leydhecker

1960

Glaukom

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Glaucoma Associated with Polycystic Kidney Disease

Cited by 5 publications

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TRPP2 is located in the primary cilia of human non-pigmented ciliary epithelial cells

TRPP2 is located in the primary cilia of human non-pigmented ciliary epithelial cells

Distribution and function of polycystin-2 in mouse retinal ganglion cells

Klinische Formen des Glaukoms und Hypothesen über die Ursachen

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