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Anti‐Neuronal Nuclear Antibody 3 Autoimmunity Targets Dachshund Homolog 1
Author(s) -
Zekeridou Anastasia,
Yang Binxia,
Len Vanda A.,
Guo Yong,
Wu Liang,
Lucchinetti Claudia F.,
McKeon Andrew,
Pittock Sean J.,
Flanagan Eoin P.
Publication year - 2022
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.26320
Subject(s) - colocalization , autoimmunity , antigen , antibody , gait ataxia , immunology , dysautonomia , medicine , immune system , ataxia , pathology , immunohistochemistry , opsoclonus , biomarker , biology , neuroscience , genetics , neuroblastoma , disease , cell culture
The antigen specificity of Anti‐Neuronal Nuclear Antibody‐type 3 (ANNA3)‐IgG is unknown. We identified Dachshund‐homolog 1 (DACH1) as the ANNA3 autoantigen and confirmed it by antigen‐specific assays, immunohistochemical colocalization and immune absorption experiments. Patients' median age was 63.5 years (range, 49–88); 67% were female. Neurological manifestations (information available for 30 patients) included one or more of neuropathy, 12; cognitive difficulties, 11; ataxia, 8; dysautonomia, 7. Evidence of a neoplasm was present in 27 of 30 (90%), most of neuroendocrine lineage. DACH1‐IgG is rare and represents a novel proposed biomarker of neurological autoimmunity and cancer. ANN NEUROL 2022;91:670–675
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