Premium
Reduced expression of BTBD10 in anterior horn cells with G olgi fragmentation and pTDP ‐43‐positive inclusions in patients with sporadic amyotrophic lateral sclerosis
Author(s) -
Furuta Natsumi,
Makioka Kouki,
Fujita Yukio,
Ikeda Masaki,
Takatama Masamitsu,
Matsuoka Masaaki,
Okamoto Koichi
Publication year - 2013
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12010
Subject(s) - amyotrophic lateral sclerosis , polyclonal antibodies , sod1 , immunohistochemistry , biology , pathology , pathogenesis , cytoplasm , microbiology and biotechnology , chemistry , antibody , medicine , immunology , disease
Overexpression of BTBD10 ( BTB / POZ domain‐containing protein 10) suppresses G93A ‐superoxide dismutase 1 ( SOD1 )‐induced motor neuron death in a cell‐based amyotrophic lateral sclerosis ( ALS ) model. In the present study, paraffin sections of spinal cords from 13 patients with sporadic ALS and 10 with non‐ ALS disorders were immunostained using a polyclonal anti‐ BTBD10 antibody. Reduced BTBD10 expression in the anterior horn cells was more frequent in spinal cords from ALS patients than in cords from patients with non‐ ALS disorders. We further investigated the relationship between the level of BTBD10 immunoreactivity and the morphology of the G olgi apparatus ( GA ) and the presence of phosphorylated TAR‐DNA ‐binding protein 43 ( pTDP ‐43). Mirror sections of spinal cords from five sporadic ALS cases were immunostained with antibodies against BTBD10 and trans‐ G olgi‐network ( TGN )‐46 or pTDP ‐43. Whereas 89.7–96.5% of the neurons with normal BTBD10 immunoreactivity showed normal GA morphology and no pTDP ‐43 cytoplasmic aggregates, 86.2–94.3% of the neurons with reduced BTBD10 expression showed GA fragmentation and abnormal pTDP ‐43 aggregates. These findings suggest that reduced BTBD10 expression is closely linked to the pathogenesis of sporadic ALS .