Cobalt chloride-simulated hypoxia elongates primary cilia in immortalized human retina pigment epithelial-1 cells

2021 ◽  
Vol 555 ◽  
pp. 190-195
Author(s):  
Ying Qiao ◽  
Zhengduo Wang ◽  
Raimonda Bunikyte ◽  
Xi Chen ◽  
Shuang Jin ◽  
...  
Keyword(s):  
Primary Cilia ◽  
Cobalt Chloride ◽  
Human Retina ◽  
Pigment Epithelial ◽  
Simulated Hypoxia

scholarly journals Characterization of USH1C/harmonin in the human retina provides insights into pathophysiology and therapy options for Usher syndrome

2021 ◽  
Author(s):  
Kerstin Nagel-Wolfrum ◽  
Benjamin R. Fadl ◽  
Mirjana M. Becker ◽  
Kirsten A. Wunderlich ◽  
Jessica Schäfer ◽  
...  
Keyword(s):  
Cell Biology ◽  
Primary Cilia ◽  
Genetic Disorder ◽  
Usher Syndrome ◽  
Photoreceptor Cells ◽  
Retinal Cell ◽  
Human Retina ◽  
Western Blots ◽  
Alternatively Spliced ◽  
Complex Genetic Disorder

AbstractHuman Usher syndrome (USH) is a complex genetic disorder that comprises three clinical subtypes USH1, USH2 and USH3 and the most common form of hereditary combined deaf-blindness. Since rodent USH1 models do not reflect the ocular phenotype observed in human patients to date only little is known about the pathophysiology of USH1 in the human eye. The USH1C gene is heavily alternatively spliced and encodes for numerous harmonin isoforms that function as scaffold proteins and organizer of the USH interactome. RNA-seq analysis of USH1C revealed harmonin a1 as the most abundant transcript of USH1C in the human retina. Bulk mRNA-seq and Western blots confirmed abundant expression of harmonin in Müller glia cells (MGCs) and retinal neurons. We located harmonin in the terminal endfeet and apical microvilli of MGCs and in photoreceptor cells (PRCs), particularly in cone synapses and outer segments of rods as well as at adhesive junctions between both, MGCs and PRCs in the outer limiting membrane (OLM). We evidenced the interaction of harmonin with OLM molecules and rhodopsin in PRCs. We correlate the identified harmonin subcellular expression and colocalization with the clinical phenotype observed in USH1C patients. Furthermore, we demonstrate a phenotype in primary cilia in patient-derived fibroblasts which we were able to revert by gene addition of harmonin a1. Our data provide novel insights for retinal cell biology, USH1C pathophysiology and subsequent gene therapy development.

scholarly journals The Proteasome: A Target of Oxidative Damage in Cultured Human Retina Pigment Epithelial Cells

2008 ◽  
Vol 49 (8) ◽  
pp. 3622 ◽  
Author(s):  
Xinyu Zhang ◽  
Jilin Zhou ◽  
Alexandre F. Fernandes ◽  
Janet R. Sparrow ◽  
Paulo Pereira ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Oxidative Damage ◽  
Human Retina ◽  
Pigment Epithelial ◽  
Pigment Epithelial Cells

Pigment epithelial ensheathment and phagocytosis of extrafoveal cones in human retina

1977 ◽  
Vol 277 (958) ◽  
pp. 459-471 ◽  
Keyword(s):  
Electron Microscopy ◽  
Outer Segment ◽  
Pigment Epithelium ◽  
Apical Surface ◽  
Human Retina ◽  
Pigment Epithelial ◽  
Transmission Electron ◽  
Intracellular Organelles ◽  
Pigment Epithelial Cells ◽  
Scanning Electron

The association between extrafoveal cone outer segments and pigment epithelial cells was studied by transmission electron microscopy in three human retinas; ages 5, 45 and 60. The pigment epithelial apical surface from a fourth human retina, age 38, was viewed in the scanning electron microscope. Multiple villous-like apical processes protrude from the pigment epithelium into the space above each cone. Sometimes one or more of these processes is sheet-like in form and contains a wealth of intracellular organelles, including mitochondria. One or more of the villous-like procesess reaches the cone and expands to ensheath the upper one-third of the outer segment. Like vertebrate rods, extrafoveal human cones shed their terminal disks in packets and these packets are phagocytosed by the ensheathing apical processes. The phagosomes then ascend in the processes toward the pigment epithelia soma. Digestion of phagosomes appears to begin in the apical processes.

scholarly journals Effect of nitric oxide on proliferation of human retina pigment epithelial cells

Eye ◽  
2000 ◽  
Vol 14 (6) ◽  
pp. 899-902 ◽  
Author(s):  
Gürsel Yilmaz ◽  
Peter Esser ◽  
Norbert Kociek ◽  
Klaus Heimann
Keyword(s):  
Nitric Oxide ◽  
Epithelial Cells ◽  
Human Retina ◽  
Pigment Epithelial ◽  
Pigment Epithelial Cells

Comparison between the effects of diallyl tetrasulfide on human retina pigment epithelial cells (ARPE-19) and HCT116 cells

2013 ◽  
Vol 1830 (11) ◽  
pp. 5267-5276 ◽  
Author(s):  
Nathaniel Edward Bennett Saidu ◽  
Imad Abu Asali ◽  
Brigitte Czepukojc ◽  
Berthold Seitz ◽  
Claus Jacob ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Human Retina ◽  
Pigment Epithelial ◽  
Pigment Epithelial Cells ◽  
Hct116 Cells
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