Animal models of immune-mediated demyelinating polyneuropathies

• Published in: Autoimmunity (Chur, Switzerland) Vol. 57; no. 1; p. 2361745
• Main Author: Ubogu, Eroboghene E
• Format: Journal Article
• Language: English
• Published: England Taylor & Francis Group 01.12.2024
• Subjects: Acute inflammatory demyelinating polyradiculoneuropathy; Adaptive Immunity; animal models; Animals; Autoimmunity; blood-nerve barrier; chronic inflammatory demyelinating polyradiculoneuropathy; demyelinating neuritis; Disease Models, Animal; experimental autoimmune neuritis; Histocompatibility Antigens Class II - immunology; Histocompatibility Antigens Class II - metabolism; Humans; Mice; Mice, Knockout; Neuritis, Autoimmune, Experimental - immunology; Polyneuropathies - etiology; Polyneuropathies - immunology; demyelinating neuritis; pathogenesis; animal models; blood-nerve barrier; chronic inflammatory demyelinating polyradiculoneuropathy; spontaneous autoimmune peripheral polyneuropathy; Acute inflammatory demyelinating polyradiculoneuropathy; major histocompatibility complex; experimental autoimmune neuritis; mice
• ISSN: 1607-842X; 0891-6934; 1607-842X
• DOI: 10.1080/08916934.2024.2361745

Immune-mediated demyelinating polyneuropathies (IMDPs) are rare disorders in which dysregulated adaptive immune responses cause peripheral nerve demyelinating inflammation and axonal injury in susceptible individuals. Despite significant advances in understanding IMDP pathogenesis guided by patient data and representative mammalian models, specific therapies are lacking. Significant knowledge gaps in IMDP pathogenesis still exist, e.g. precise antigen(s) and mechanisms that initially trigger immune system activation and identification of large population disease susceptibility factors. The initial directional cues for antigen-specific effector or autoreactive leukocyte trafficking into peripheral nerves are also unknown. An overview of current animal models, with emphasis on the experimental autoimmune neuritis and spontaneous autoimmune peripheral polyneuropathy models, is provided. Insights on the initial directional cues for peripheral nerve tissue specific autoimmunity using a novel Major Histocompatibility Complex class II conditional knockout mouse strain are also discussed, suggesting an essential research tool to study cell- and time-dependent adaptive immunity in autoimmune diseases.