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PU.1, IKAROS, E2A, EBF, and PAX5 comprise a transcriptional network that orchestrates B-cell lineage specification, commitment, and differentiation. Here we identify interferon regulatory factor 8 (IRF8) as another component of this complex, and show that it also modulates lineage choice by hematopoietic stem cells (HSCs). IRF8 binds directly to an IRF8/Ets(More)
We generated a mouse line in which the src homology 2 domain-bearing protein tyrosine phosphatase (SHP)-2 binding site of gp130, tyrosine 759, was mutated to phenylalanine (gp130(F759/F759)). The gp130(F759/F759) mice developed rheumatoid arthritis (RA)-like joint disease. The disease was accompanied by autoantibody production and accumulated(More)
The peripheral hypoglossal nerves in 13 cases of amyotrophic lateral sclerosis (ALS) and five control cases were examined using morphometrical methods to demonstrate the degenerative process of motor nerve degeneration. The total number of myelinated fibers and their histograms were analyzed according to the degree of severity of the degeneration. Reduction(More)
Muscle biopsies of 11 patients suffering from amyotrophic lateral sclerosis (ALS) were examined and the i.m. nerves found in seven of them were examined by electron microscopy. In atrophied muscles there was a marked decrease of myelinated fibers. The ultrastructure of the remaining myelinated axons showed changes in the neurofilaments, mitochondria, and(More)
Inclusions, ultrastructurally identical with Hirano bodies which were previously believed to be limited to the central nervous system (CNS), were found both within peripheral myelinated nerve axons and within terminal axons of neuromuscular junctions in the ocular muscles of an autopsied woman who had suffered from progressive external ophthalmoplegia with(More)
Joseph's disease is a hereditary ataxia found among descendants of Portuguese from the Azores Islands. We describe the clinical and pathological features of 4 members of a Japanese family who were diagnosed as having Joseph's disease. The illness began with cerebellar ataxia between the ages of 18 and 45 years. Nystagmus, dysarthria, and pyramidal signs(More)
The central nervous system (CNS) is considered an immune-privileged tissue protected by a specific vessel structure, the blood-brain barrier (BBB). Upon infection or traumatic injury in the CNS, the BBB is breached, and various immune cells are recruited to the affected area. In the case of autoimmune diseases in the CNS like multiple sclerosis (MS),(More)
KDEL receptors are responsible for retrotransporting endoplasmic reticulum (ER) chaperones from the Golgi complex to the ER. Here we describe a role for KDEL receptor 1 (KDELR1) that involves the regulation of integrated stress responses (ISR) in T cells. Designing and using an N-ethyl-N-nitrosourea (ENU)-mutant mouse line, T-Red (naïve T-cell reduced), we(More)
The central nervous system (CNS) is an immune-privileged environment protected by the blood-brain barrier (BBB), which consists of specific endothelial cells that are brought together by tight junctions and tight liner sheets formed by pericytes and astrocytic end-feet. Despite the BBB, various immune and tumor cells can infiltrate the CNS parenchyma, as(More)
A case of isolated vagus nerve palsy with herpes zoster was reported. A 31-year-old woman was admitted to our hospital with six days' history of difficulty of swallowing of fluid and hoarseness with a painful vesicle on the right ear. Neurological examination revealed poor elevation of soft palate on the right side, but the pharyngeal reflex was preserved.(More)