title: Reduced expression of BTBD10 in anterior horn cells with Golgi fragmentation and pTDP-43-positive inclusions in patients with sporadic amyotrophic lateral sclerosis.
abstract: 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 Golgi 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-Golgi-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.
authors: Department of Neurology, Gunma University Graduate School of Medicine, Gunma, Japan. n-furuta@gunma-u.ac.jp
authors: Natsumi
authors: N
authors: Furuta
authors: Kouki
authors: K
authors: Makioka
authors: Yukio
authors: Y
authors: Fujita
authors: Masaki
authors: M
authors: Ikeda
authors: Masamitsu
authors: M
authors: Takatama
authors: Masaaki
authors: M
authors: Matsuoka
authors: Koichi
authors: K
authors: Okamoto
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism* / Q000473:pathology*; D000870:Anterior Horn Cells / Q000378:metabolism* / Q000473:pathology; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D006056:Golgi Apparatus / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism* / Q000473:pathology; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D008875:Middle Aged; D009687:Nuclear Proteins / Q000096:biosynthesis*
chemical_list: C494432:BTBD10 protein, human; D004268:DNA-Binding Proteins; D047908:Intracellular Signaling Peptides and Proteins; D009687:Nuclear Proteins
keywords: ALS; BTBD10; Golgi apparatus; pTDP-43; pathology