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Spinal manifestations of CLN1 disease start during the early postnatal period
Author(s) -
Nelvagal H. R.,
Dearborn J. T.,
Ostergaard J. R.,
Sands M. S.,
Cooper J. D.
Publication year - 2021
Publication title -
neuropathology and applied neurobiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.538
H-Index - 95
eISSN - 1365-2990
pISSN - 0305-1846
DOI - 10.1111/nan.12658
Subject(s) - spinal cord , astrocytosis , medicine , neuroscience , pathology , microglia , biology , central nervous system , immunology , inflammation
The spinal cord appears especially vulnerable in CLN1 disease, a fatal inherited lysosomal storage disorder. Our data reveal a much earlier onset of spinal cord disease and accompanying behavioural changes in CLN1 mice, while spinal maturation is still ongoing. Our multi‐disciplinary data provide new insights into the spatio‐temporal staging of CLN1 pathogenesis during ongoing postnatal maturation, and highlight the need to deliver therapies during the presymptomatic period.