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NF1

Clinicopathological and Genomic Characterization of BCORL1-Driven High-Grade Endometrial Stromal Sarcomas

[Modern Pathology] The authors described 12 BCORL1-altered uterine sarcomas with striking resemblance to BCOR-altered endometrial stromal sarcoma.

Patient-Derived iPSC-Cerebral Organoid Modeling of the 17q11.2 Microdeletion Syndrome Establishes CRLF3 as a Critical Regulator of Neurogenesis

[Cell Reports] Using patient-derived human induced pluripotent stem cell-forebrain cerebral organoids (hCOs), researchers identified both neural stem cell proliferation and neuronal maturation abnormalities in neurofibromatosis type 1-total gene deletion hCOs.

Metalloproteinase 1 Downregulation in Neurofibromatosis 1: Therapeutic Potential of Antimalarial Hydroxychloroquine and Chloroquine

[Cell Death & Disease] Researchers analyzed the collagen and matrix metalloproteinase 1 (MMP1) expression in Neurofibromatosis 1 cutaneous neurofibroma and found excessive expression of collagen and reduced expression of MMP1.

Oncogenic KRAS Engages an RSK1/NF1 Pathway to Inhibit Wild-Type RAS Signaling in Pancreatic Cancer

[Proceedings of the National Academy of Sciences of the United States of America] Investigators used proximity labeling to identify protein interactors of active KRAS in pancreatic ductal adenocarcinoma (PDAC) cells. We expressed fusions of wild-type (WT) (BirA-KRAS4B), mutant (BirA-KRAS4BG12D), and nontransforming cytosolic double mutant (BirA-KRAS4BG12D/C185S) KRAS with the BirA biotin ligase in murine PDAC cells.

Metalloproteinase 1 Downregulation in Neurofibromatosis 1: Therapeutic Potential of Antimalarial Hydroxychloroquine and Chloroquine

[Cell Death & Disease] Researchers analyzed the collagen and matrix metalloproteinase 1 (MMP1) expression in Neurofibromatosis 1 cutaneous neurofibroma and found excessive expression of collagen and reduced expression of MMP1.

Stem-Like Cells Drive NF1-Associated MPNST Functional Heterogeneity and Tumor Progression

[Cell Stem Cell] Investigators discovered and characterized a rare malignant peripheral nerve sheath tumor (MPNST) cell population with stem-cell-like properties, including quiescence, that was essential for tumor initiation and relapse.

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