Details

Autor(en) / Beteiligte

• Bennett, Jeffrey L
• OʼConnor, Kevin C
• Bar-Or, Amit
• Zamvil, Scott S
• Hemmer, Bernhard
• Tedder, Thomas F
• von Büdingen, H.-Christian
• Stuve, Olaf
• Yeaman, Michael R
• Smith, Terry J
• Stadelmann, Christine

Titel

B lymphocytes in neuromyelitis optica

Ist Teil von

• Neurology : neuroimmunology & neuroinflammation, 2015-06, Vol.2 (3), p.e104-e104

Ort / Verlag

United States: American Academy of Neurology

Erscheinungsjahr

2015

Quelle

Free E-Journal (出版社公開部分のみ）

Beschreibungen/Notizen

• Neuromyelitis optica (NMO) is an inflammatory autoimmune disorder of the CNS that predominantly affects the spinal cord and optic nerves. A majority (approximately 75%) of patients with NMO are seropositive for autoantibodies against the astrocyte water channel aquaporin-4 (AQP4). These autoantibodies are predominantly IgG1, and considerable evidence supports their pathogenicity, presumably by binding to AQP4 on CNS astrocytes, resulting in astrocyte injury and inflammation. Convergent clinical and laboratory-based investigations have indicated that B cells play a fundamental role in NMO immunopathology. Multiple mechanisms have been hypothesizedAQP4 autoantibody production, enhanced proinflammatory B cell and plasmablast activity, aberrant B cell tolerance checkpoints, diminished B cell regulatory function, and loss of B cell anergy. Accordingly, many current off-label therapies for NMO deplete B cells or modulate their activity. Understanding the role and mechanisms whereby B cells contribute to initiation, maintenance, and propagation of disease activity is important to advancing our understanding of NMO pathogenesis and developing effective disease-specific therapies.