Plexiform Neurofibromas
Plexiform Neurofibromas - Related Videos
03:53
An Orthotopic Sciatic Nerve Xenograft for Neurofibromatosis Type 1 Neurofibromas
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2025
Neurofibromatosis type 1 (NF1) leads to benign peripheral nerve sheath tumors (neurofibromas) that require a specific nerve microenvironment for growth. An optimized orthotopic xenograft model was developed, using intraneural injection of human Schwann cells into NSG mice. This model recapitulates human neurofibromas and provides a valuable platform to evaluate therapeutic strategies.
02:56
Modeling Retinal Degeneration and Regeneration in Zebrafish Using a Focal Laser Injury
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2025
This video demonstrates a procedure to induce retinal degeneration in adult zebrafish using laser injury. The laser targets photoreceptor cells in the retina, triggering cell death and a degenerative process. This damage activates Müller glia (MG) cells, leading to their dedifferentiation into stem cell progenitors that aid in retinal regeneration.
06:27
Müller Glia Cell Activation in a Laser-induced Retinal Degeneration and Regeneration Model in Zebrafish
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Cited by 13 •
2017
The zebrafish is a popular animal model to study mechanisms of retinal degeneration/regeneration in vertebrates. This protocol describes a method to induce localized injury disrupting the outer retina with minimal damage to the inner retina. Subsequently, we monitor in vivo the retinal morphology and the Müller glia response throughout retinal regeneration.
07:53
Split Retina as an Improved Flatmount Preparation for Studying Inner Nuclear Layer Neurons in Vertebrate Retina
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2024
This work presents an alternative flatmount retina preparation in which the removal of photoreceptor cell bodies enables faster antibody diffusion and improved patch pipette access to inner retinal neurons for immunohistochemistry, in situ hybridization, and electrophysiology experiments.
07:48
Organotypic Hippocampal Slice Cultures As a Model to Study Neuroprotection and Invasiveness of Tumor Cells
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Cited by 18 •
2017
Organotypic hippocampal slice cultures (OHSC) represent an in vitro model that simulates the in vivo situation very well. Here we describe a vibratome-based improved slicing protocol to obtain high quality slices for use in assessing the neuroprotective potential of novel substances or the biological behavior of tumor cells.
FAQs
Loss-of-function changes in the NF1 gene impair production or activity of neurofibromin, a regulator of RAS signaling. When this regulatory control is disrupted, Schwann cells and other associated nerve-tissue cells can proliferate abnormally. This molecular link connects an inherited or acquired genetic change with tumor development and provides a basis for investigating pathway-directed treatments.
Schwann cells are central to the abnormal cellular proliferation associated with these tumors, while surrounding nerve-tissue cells and the peripheral nerve environment also contribute to tumor behavior. Studying these nerve–tumor interactions helps researchers examine how tumors begin, expand along nerve branches, and alter their local biological setting rather than viewing tumor cells in isolation.
Transformation into malignant peripheral nerve sheath tumors represents a clinically important progression that researchers seek to understand and detect. Plexiform neurofibromas therefore support studies of disease progression and cancer risk, including the biological changes that may accompany increasing malignancy. This relationship makes them useful for investigating how a nerve-associated tumor can evolve into a more aggressive cancer.
Evaluation combines a physical examination with magnetic resonance imaging and molecular analysis. The examination assesses the patient directly, imaging helps characterize tumor involvement within the body, and molecular analysis examines relevant biological features. Using these approaches together gives clinicians and researchers complementary information for studying tumor burden, disease behavior, and potential progression.
Magnetic resonance imaging contributes an anatomical view that complements physical examination and molecular analysis. Because plexiform neurofibromas can extend along multiple peripheral-nerve branches, imaging is part of the combined evaluation used to characterize their distribution and involvement. In research, this information supports assessment of disease progression and the effects of approaches intended to reduce tumor growth.
Targeted pathway inhibitors are being studied because NF1-related disruption of neurofibromin affects RAS signaling. These treatments aim to interfere with pathway activity that supports abnormal cell proliferation, with the intended outcomes of reducing tumor growth, easing symptoms, and lowering cancer risk. Their investigation links molecular mechanism to therapeutic development in cancer research.
These tumors provide a model that connects several cancer research questions: tumor initiation, nerve–tumor interactions, disease progression, and transformation into malignant peripheral nerve sheath tumors. Their association with NF1 also allows investigators to relate gene regulation and RAS signaling to tumor behavior. Findings from this model can inform studies of targeted inhibition and cancer risk.