scholarly journals RB1 gene inactivation by chromothripsis in human retinoblastoma

Oncotarget ◽  
2014 ◽  
Vol 5 (2) ◽  
pp. 438-450 ◽  
Author(s):  
Justina McEvoy ◽  
Panduka Nagahawatte ◽  
David Finkelstein ◽  
Jennifer Richards-Yutz ◽  
Marcus Valentine ◽  
...  
2002 ◽  
Vol 20 (5) ◽  
pp. 408-408 ◽  
Author(s):  
Kwong Wai Choy ◽  
Chi Pui Pang ◽  
Christopher B.O. Yu ◽  
Hing Lok Wong ◽  
Joan S.K. Ng ◽  
...  

Cancers ◽  
2021 ◽  
Vol 13 (20) ◽  
pp. 5248
Author(s):  
Swatishree Sradhanjali ◽  
Padmalochan Rout ◽  
Devjyoti Tripathy ◽  
Swathi Kaliki ◽  
Suryasnata Rath ◽  
...  

Retinoblastoma is usually initiated by biallelic RB1 gene inactivation. In addition, MYCN copy number alterations also contribute to RB pathogenesis. However, MYCN expression, its role in disease progression and correlation with RB histological risk factors are not well understood. We studied the expression of MYCN in enucleated RB patient specimens by immunohistochemistry. MYCN is overexpressed in RB compared to control retina. Our microarray gene expression analysis followed by qRT-PCR validation revealed that genes involved in glucose metabolism and migration are significantly downregulated in MYCN knockdown cells. Further, targeting MYCN in RB cells using small molecule compounds or shRNAs led to decreased cell survival and migration, increased apoptosis and cell cycle arrest, suggesting that MYCN inhibition can be a potential therapeutic strategy. We also noted that MYCN inhibition results in reduction in glucose uptake, lactate production, ROS levels and gelatinolytic activity of active-MMP9, explaining a possible mechanism of MYCN in RB. Taking clues from our findings, we tested a combination treatment of RB cells with carboplatin and MYCN inhibitors to find enhanced therapeutic efficacy compared to single drug treatment. Thus, MYCN inhibition can be a potential therapeutic strategy in combination with existing chemotherapy drugs to restrict tumor cell growth in RB.


1999 ◽  
Vol 30 (6) ◽  
pp. 462-478
Author(s):  
P J Venta ◽  
Y Cao ◽  
L Alexander ◽  
V Yuzbasiyan-Gurkan

2018 ◽  
Vol 18 (6) ◽  
pp. 875-881 ◽  
Author(s):  
Xue Zhu ◽  
Ke Wang ◽  
Kai Zhang ◽  
Yi Pan ◽  
Fanfan Zhou ◽  
...  

Background: Retinoblastoma is the most common intraocular malignant tumor in childhood. Although external beam radiation and enucleation are effective to control retinoblastoma, eye salvage and vision preservation are still significant challenges. Polyphyllin I (PPI), a natural compound extracted from Paris polyphylla rhizomes, has a wide range of activities against many types of cancers. However, the potential effect of this herbal compound on retinoblastoma has not yet been investigated. Method: In the present study, we evaluated the cytotoxic effect of PPI on human retinoblastoma Y-79 cells as well as its underlying molecular mechanism. Our results indicated that PPI treatment significantly inhibited cell proliferation, arrested the cell cycle at G2/M phase and induced cell apoptosis of Y79 cells through the mitochondrial- dependent intrinsic pathway. Moreover, p53 is involved in PPI-induced cytotoxicity in human retinoblastoma Y-79 cells. Exposure to 10 μM PPI for 48 h dramatically induced the expression levels of p53, phosphorylated- p53 and acetylated-p53. Furthermore, blockade of p53 expression effectively attenuated PPI-induced cell cycle arrest and cell apoptosis in Y-79 cells. Result: These results demonstrated that PPI exhibits anti-proliferation effect on human retinoblastoma Y-79 cells through modulating p53 expression, stabilization and activation. This information shed light on the potential application of PPI in retinoblastoma therapy.


BIO-PROTOCOL ◽  
2016 ◽  
Vol 6 (15) ◽  
Author(s):  
Kangming Chen ◽  
Huilan Zhu ◽  
Liping Gu ◽  
Shengni Tian ◽  
Ruanbao Zhou

2005 ◽  
Vol 217 (2) ◽  
pp. 213-219 ◽  
Author(s):  
Temduang Limpaiboon ◽  
Prasong Khaenam ◽  
Patcharee Chinnasri ◽  
Montisha Soonklang ◽  
Patcharee Jearanaikoon ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document