The structural properties of normal-type spinel ZnCr 2 Se 4 have been studied as a function of temperature by means of X-ray diffraction using synchrotron radiation. It is found that a structural phase transition from cubic to orthorhombic symmetry occurs at 22.5 K (T c ). It is also found that, below T c , Se ions are cooperatively displaced in a pseudo-tetragonal (001) plane. Since the Cr 3+ ion has no orbital angular momentum, a CrSe 6 octahedron is not distorted by a crystal field produced by surrounding Se 2− anions. It is interpreted that the pseudo-tetragonal displacements of Se ions are induced by magnetostriction among Cr 3+ ions in CrSe 2 chains, in which Cr ions share two Se 2− with each other along 〈110〉.
Huntington's disease (HD) is an inherited neurodegenerative disease caused by a polyglutamine repeat expansion in the huntingtin protein. Immunohistochemical studies using the 1C2 antibody for polyglutamine expansion have detected characteristic intranuclear inclusions (INIs) in affected neurons in HD. Further, in vitro and mouse models of HD have shown that the INIs recruit several proteins relating to RNA splicing and translation. In the present study, we immunohistochemically investigated the association of INIs with various heterogeneous nuclear ribonucleoproteins in the cerebral cortex of four autopsy cases of HD. Fused in sarcoma (FUS) was colocalized with 1C2‐positive nuclear inclusions in all examined cases. Localization of poly (rC)‐binding protein 1 (PCBP1) in 1C2‐positive nuclear inclusions was also observed. Double immunofluorescence revealed complete or partial loss of the normal, diffuse nuclear distribution of FUS or PCBP1 in neurons with 1C2‐positive nuclear inclusions. This maldistribution of FUS in cortical neurons suggests a severe disturbance of messenger RNA processing, which may be a common pathogenetic mechanism of FUS‐related familial amyotrophic lateral sclerosis.
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