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Case of uveitis with increased electroretinographic amplitudes following Nivolumab and Ipilimumab administration for malignant melanoma.
Doc Ophthalmol
March 2025
Department of Ophthalmology, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan.
Purpose: To report our flicker electroretinographic (ERG) findings in a patient who developed uveitis after treatment with immune checkpoint inhibitors (ICIs) for a metastatic malignant melanoma.
Methods: ERGs were used to monitor retinal physiology in a patient with ocular complications following systemic ICI administration. Flicker ERGs were recorded using the RETeval system before and after the ICI treatments.
Cutaneous Hemangioma With Epithelioid Features Harboring TPM3/4::ALK Fusions: A Distinct Entity or a Molecular Variant of Epithelioid Hemangioma?
Am J Surg Pathol
March 2025
Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
Vascular neoplasms with epithelioid cytomorphology encompass a wide spectrum of benign and malignant lesions, including epithelioid hemangioma (EH), cutaneous epithelioid angiomatous nodule (CEAN), epithelioid hemangioendothelioma (EHE), and epithelioid angiosarcoma (EAS). Recently, the first case of a cutaneous hemangioma with epithelioid features harboring a TPM3::ALK fusion was reported. Herein, we report 4 additional cases, including 1 case with an alternate TPM4::ALK fusion, and expand on the clinicopathologic and molecular genetic features of these unusual vascular lesions.
View Article and Find Full Text PDFSLC37A3-associated retinitis pigmentosa: a case report of clinical features and three-year follow up.
Doc Ophthalmol
March 2025
Department of Ophthalmology, Vanderbilt Clinic, Columbia University Irving Medical Center, 622 W 168th St 3rd Floor, New York, NY, 10032, USA.
Purpose: To describe a case of SLC37A3-associated retinitis pigmentosa (RP) and associated imaging and electroretinography findings.
Methods: The patient was evaluated at Columbia University Irving Medical Center using a comprehensive multimodal imaging protocol that included color fundus photography, fundus autofluorescence (FAF), and spectral-domain optical coherence tomography. Functional assessments were conducted using full-field electroretinography (ERG), following the ISCEV standard protocols to ensure consistent and reproducible measurements of photoreceptor activity.
Rational design of cyclic peptides, with an emphasis on bicyclic peptides.
Curr Opin Struct Biol
March 2025
BicycleTx Ltd, Blocks A&B, Portway Building, Granta Park, Great Abington, Cambridge, CB21 6GS, UK.
Macrocyclic peptides are a promising chemotype for drug discovery, given their attractive properties of proteolytic stability, bioavailability and the ability to inhibit protein-protein interactions. Approaches to the generation of macrocyclic peptides include optimisation of hits from library screening; de novo design from known ligands and antibody paratopes or protein-protein interactions; constraint of linear peptides to afford beneficial properties of macrocycles; and novel approaches to cyclisation. We describe the recent literature and exemplify these approaches in the design of peptide macrocycles, and the benefits of incorporating computational and structure-guided approaches into compound design and optimisation.
View Article and Find Full Text PDFLoss of the vitamin A receptor RBPR2 in mice disrupts whole-body retinoid homeostasis and the quantitative balance regulating retinylidene protein synthesis.
FASEB J
March 2025
Department of Ophthalmology, University of Minnesota, Minneapolis, Minnesota, USA.
The distribution of stored dietary vitamin A/all-trans-retinol (ROL) from the liver throughout the body is critical for maintaining retinoid function in peripheral tissues and for generating visual pigments for photoreceptor cell function. ROL circulates in the blood bound to the retinol binding protein 4 (RBP4) as RBP4-ROL. Two membrane receptors, RBPR2 in the liver and other non-ocular tissues, and STRA6 in the eye are proposed to bind circulatory RBP4 and this mechanism facilitates the internalization of ROL.
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