Publications (3)5.43 Total impact
Article: Ubiquitin-related proteins in neuronal and glial intranuclear inclusions in intranuclear inclusion body disease.[show abstract] [hide abstract]
ABSTRACT: Recent studies have shown that eosinophilic intranuclear inclusions (INI) in the brain of patients with intranuclear inclusion body disease (INIBD) are immunopositive for ubiquitin and ubiquitin-related proteins (URP). However, the extent and frequency of URP-immunoreactive inclusions in INIBD are uncertain. We immunohistochemically examined the brain, spinal cord and dorsal root ganglia from five patients with INIBD, using a virtual slide system with sequential staining of the same sections with hematoxylin and eosin and by immunolabeling with antibodies against ubiquitin and URP (NEDD8, NUB1, SUMO-1 and SUMO-2). Intranuclear inclusions were widely distributed in neurons and glial cells in all the cases. Sequential staining revealed that 100% of INI in neurons and glial cells were positive for ubiquitin. Moreover, the majority or a significant proportion of INI were positive for NEDD8, NUB1, SUMO-1 and SUMO-2. However, the proportions of NEDD8-, NUB1- and SUMO-1-positive inclusions were significantly higher in neurons than in glial cells (P < 0.05). These findings suggest that proteins related to ubiquitination and proteasomal degradation are involved in the formation of INI in INIBD.Pathology International 06/2012; 62(6):407-11. · 1.62 Impact Factor
Article: FUS immunoreactivity of neuronal and glial intranuclear inclusions in intranuclear inclusion body disease.[show abstract] [hide abstract]
ABSTRACT: Recent studies have shown that fused-in-sarcoma (FUS) protein is a component of 'neuronal' intranuclear inclusion bodies (INIBs) in the brains of patients with intranuclear inclusion body disease (INIBD). However, the extent and frequency of FUS-immunoreactive structures in INIBD are uncertain. We immunohistochemically examined the brain, spinal cord and peripheral ganglia from five patients with INIBD and five control subjects, using anti-FUS antibodies. In controls, the nuclei of both neurones and glial cells were intensely immunolabelled with anti-FUS and neuronal cytoplasm was weakly positive for FUS. In INIBD, neuronal and glial INIBs in the brain and spinal cord were positive for FUS. FUS-positive INIBs were also found in the peripheral ganglia. The proportion of FUS-positive neuronal INIBs relative to the total number of inclusion-bearing neurones ranged from 55.6% to 83.3% (average 73.2%) and that of FUS-positive glial INIBs ranged from 45.9% to 85.7% (average 62.7%). The nucleus and cytoplasm of inclusion-bearing neurones and glial cells showed no FUS immunoreactivity. These findings suggest that FUS is incorporated into INIBs in both neurones and glial cells and that loss of normal FUS immunoreactivity may result from reduced protein expression and/or sequestration within inclusions.Neuropathology and Applied Neurobiology 08/2011; 38(4):322-8. · 3.80 Impact Factor
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ABSTRACT: A 38-year-old man with non-Hodgkin's lymphoma presented with hypesthesia and muscle weakness in the left upper limb. A lack of F-waves in left median and ulnar nerve conduction studies suggested a lesion at the proximal segments of the peripheral nerves, such as the left brachial plexus or nerve roots. Cervical magnetic resonance imaging revealed no lesions compressing nerve roots or peripheral nerves. Small and obscure uptake on the left side of the cervical nerve roots on 67Ga-scintigraphy was indistinguishable from artifact. Positron emission tomography-computed tomography (PET/CT) revealed a region of high glucose uptake in a left cervical intervertebral foramen, leading to a diagnosis of neurolymphomatosis. Neurological symptoms improved following additional chemotherapy, and the high glucose-uptake lesion disappeared. FDG-PET/CT is useful for rapid and non-invasive evaluation of neurolymphomatosis.Rinsho shinkeigaku = Clinical neurology 08/2007; 47(7):437-40.