Magnesium-dependent-Protein Phosphatase 1B Regulates the Protein Arginine Methyltransferase 5 Through the Modulation of Myosin Phosphatase.

Dysregulation of the expression levels and the activity of kinases/phosphatases is an intrinsic hallmark of tumor transformation and progression, as either as a primary cause or consequence. The myosin phosphatase (MP)/protein arginine methyltransferase 5 (PRMT5)/histone (H4) pathway is an oncogenic signaling pathway downregulating the gene expression of tumor suppressors. However, the upstream regulators of the pathway are unknown.

The role of Intravitreal chemotherapy as an adjunctive treatment for retinoblastoma: a systematic review and single-arm meta-analysis.

Topic: evaluation of clinical outcomes of patients with retinoblastoma treated with intravitreal chemotherapy (IvitC).

Design: Systematic review and single-arm meta-analysis CLINICAL RELEVANCE: Clinical outcomes with IVitC vary across reports according to patient characteristics and concomitant treatment modalities, mainly intravenous chemotherapy (IVC) and intra-arterial chemotherapy (IAC). There are currently no large clinical trials or meta-analyses focusing on the topic.

In vitro model of retinoblastoma derived tumor and stromal cells for tumor microenvironment (TME) studies.

Retinoblastoma (RB) is an intraocular tumor arising from retinal cone progenitor cells affecting young children. In the last couple of years, RB treatment evolved towards eye preserving therapies. Therefore, investigating intratumoral differences and the RB tumor microenvironment (TME), regulating tumorigenesis and metastasis, is crucial.

Clinical and genetic characterization of a progressive RBL2-associated neurodevelopmental disorder.

Retinoblastoma (RB) proteins are highly conserved transcriptional regulators that play important roles during development by regulating cell-cycle gene expression. RBL2 dysfunction has been linked to a severe neurodevelopmental disorder. However, to date, clinical features have only been described in six individuals carrying five biallelic predicted loss of function (pLOF) variants.

[TREATMENT UPDATES IN OCULAR ONCOLOGY].

Malignant tumors of the eye are uncommon, yet of great importance due to their effect on the patient's life and vision. The most common primary intraocular tumors are retinoblastoma in children and uveal melanoma in adults. The most common periocular tumor is basal cell carcinoma in eyelids.

Anti‑proliferative effects of in a model for triple negative breast cancer.

Triple negative breast cancer (TNBC) is characterized by the absence of hormones and growth factor receptors. It is typically responsive to anthracycline/taxol-based conventional chemotherapy. However, major therapeutic limitations include systemic toxicity and acquired resistance to chemotherapeutics.

Bilateral familial retinoblastoma diagnosed via optical coherence tomography following a normal funduscopic exam.

Retinoblastoma (RB) is the most common intraocular malignancy in children, and patients with family history of retinoblastoma are at high risk of disease. While intensive screening programs have led to earlier diagnosis and higher rates of globe salvage, visual outcomes have not improved. Handheld optical coherence tomography (HH-OCT) is a non-invasive imaging modality that can be utilized for screening, diagnosis, and monitoring of Familial RB.

RB functions as a key regulator of senescence and tumor suppression.

The Retinoblastoma (RB) protein is crucial for regulating gene transcription and chromatin remodeling, impacting cell cycle progression, cellular senescence, and tumorigenesis. Cellular senescence, characterized by irreversible growth arrest and phenotypic alterations, serves as a vital barrier against tumor progression and age-related diseases. RB is crucial in mediating senescence and tumor suppression by modulating the RB-E2F pathway and cross talking with other key senescence effectors such as p53 and p16INK4a.

Non-RB1 germline cancer predisposing variants found in retinoblastoma patients.

Purpose: It is well known that individuals with hereditary retinoblastoma are at lifelong high risk for developing subsequent malignant neoplasms (SMN). However, the role that non- germline variants play in tumorigenesis and SMN risk has not yet been studied. The purpose of this study is to report the frequency and spectrum of non- germline cancer predisposing variants in individuals with retinoblastoma (RB).

Reading level, acuity, and speed evaluation among retinoblastoma survivors: A prospective case series.

Pediatric retinoblastoma survivors exhibit visual deficits. How these visual deficits impact reading skills is unknown. The purpose of this study is to assess reading level, reading acuity, and reading speed among retinoblastoma survivors.

The significance of in multiple myeloma.

The treatment of multiple myeloma (MM) has significantly advanced; however, the underlying genetic mechanisms remain elusive. Clonal events and genetic alterations are recognized as pivotal in the pathogenesis of MM. It is now understood that a multitude of gene mutations, including those affecting RAS, , , and 1q21 amplification, are prevalent in this disease.

Age distribution of retinoblastoma tumours in familial disease.

Background: 40% of children with retinoblastoma have an RB1 gene mutation identified, known as heritable retinoblastoma. It is important to undertake active surveillance (screening) of relatives of those with identified RB1 gene mutations and ensure ongoing surveillance to monitor for new tumour formation or recurrences. Current guidance is to screen patients up to the age of 7 years old.

Actin-based deformations of the nucleus control mouse multiciliated ependymal cell differentiation.

Ependymal cells (ECs) are multiciliated cells in the brain that contribute to cerebrospinal fluid flow. ECs are specified during embryonic stages but differentiate later in development. Their differentiation depends on genes such as GEMC1 and MCIDAS in conjunction with E2F4/5 as well as on cell-cycle-related factors.

Spotlighting Turnover Costs for Pediatric Ocular Oncology Exams under Anesthesia.

Introduction: While pediatric intraocular malignancies are rare, management requires frequent examinations under anesthesia (EUAs) which are financial costly. The purpose of the study was to measure operating room (OR) turnover costs and to estimate potential cost savings of reducing turnover time.

Methods: Turnover time data for EUAs with a single ocular oncology provider at the University of Michigan were analyzed from 2021 to 2023.

Intraocular Surgery for Retinoblastoma: An Evaluation of Current Evidence.

Background: Retinoblastoma (Rb) is the most common intraocular malignancy of childhood. The prognosis in Rb directly relates to the spread of disease beyond the eye, particularly to the central nervous system. Therefore, until the recent past, surgically entering an eye with active or recently active Rb was absolutely contraindicated in most centres around the world due to the risk of iatrogenic extraocular spread.

Retinal organoid chip: engineering a physiomimetic oxygen gradient for optimizing long term culture of human retinal organoids.

An oxygen gradient across the retina plays a crucial role in its development and function. The inner retina resides in a hypoxic environment (2% O) adjacent to the vitreous cavity. Oxygenation levels rapidly increase towards the outer retina (18% O) at the choroid.

Mimicking Retinoblastoma Treatment With Repeated Topotecan or Melphalan Develops Cross-Resistance to Classic Agents But Not to Repurposed Drugs.

Purpose: Refractory or recurrent retinoblastoma results from acquired chemoresistance and the management of these eyes often requires surgical removal. Our objective was to develop retinoblastoma models resistant to chemotherapy by exposing cancer cells to repeated chemotherapy mimicking the clinical scenario. These newly resistant cells were used to evaluate potential novel therapies.

Precision Control of Cell Type-Specific Behavior via RNA Sensing and Editing.

In the realms of bioengineering and biopharmaceuticals, there exists a critical demand for advanced genetic tools that can interact with specific cell signaling pathways to accurately identify and target various cell types. This research introduces the innovative CRISPR-ADAReader system, which enables precise manipulation of cell activity through sensing target RNA. Featuring both positive and negative feedback loops, the system allows for tailored regulation across different cell types in response to various internal signals, showcasing exceptional programmability, specificity, and sensitivity.