• Neural crest cells are a temporary group of cells that arise from the embryonic ectoderm germ layer, and in turn give rise to a diverse cell lineage-including melanocytes, craniofacial cartilage and bone, smooth muscle, peripheral and enteric neurons and glia. (wikipedia.org)
  • HIV may act directly by infecting dorsal root ganglion neurons. (medscape.com)
  • Autonomic dysfunction is common in HIV infection and is associated with distal symmetric polyneuropathy. (medscape.com)
  • Anti-Caspr2 encephalitis (Morvan's syndrome) is associated with limbic symptoms, severe autonomic disorders, muscle cramps and burning extremity pain due to peripheral nerve hyperexcitability. (bvsalud.org)
  • HIV induces muscle fibers to express major histocompatibility complex-1 (MHC-1) on their surface, triggering T cell-mediated muscle fiber injury. (medscape.com)
  • Anti-Caspr2 antibodies bind to Caspr2 on the surface of afferent cells in the dorsal root ganglion, and internalization of VGKCs causes a decrease in the K+ current, resulting in neuronal hyperexcitation and severe pain. (bvsalud.org)
  • Underlying the development of neural crest is a gene regulatory network, described as a set of interacting signals, transcription factors, and downstream effector genes that confer cell characteristics such as multipotency and migratory capabilities. (wikipedia.org)
  • This study reports the generation of a new mouse model of GAN based on overexpression of Prph in the context of targeted disruption of gigaxonin gene. (bvsalud.org)
  • Giant axonal neuropathy is a pathological condition caused by mutations in the GAN gene that encodes gigaxonin. (bvsalud.org)
  • Disease-causing variants in the gigaxonin gene (GAN) cause autosomal recessive giant axonal neuropathy. (bvsalud.org)
  • BACKGROUND: Giant axonal neuropathy (GAN) is a progressive childhood hereditary polyneuropathy that affects both the peripheral and central nervous systems. (bvsalud.org)
  • This new mouse model should be useful to investigate the pathogenic changes associated with GAN disease and for drug testing.SIGNIFICANCE STATEMENT Research on pathogenic mechanism and treatment of GAN has been hampered by the lack of animal models exhibiting overt phenotypes and substantial neurofilament disorganization, a hallmark of the disease. (bvsalud.org)
  • Autonomic dysfunction is common in HIV infection and is associated with distal symmetric polyneuropathy. (medscape.com)
  • At 12 months of age, the Gan-/-;TgPer mice exhibited cognitive deficits as well as severe sensory and motor defects. (bvsalud.org)