Park, Hongsun published the artcileHornerin deposits in neuronal intranuclear inclusion disease: direct identification of proteins with compositionally biased regions in inclusions, SDS of cas: 63-68-3, the publication is Acta Neuropathologica Communications (2022), 10(1), 28, database is CAplus and MEDLINE.
Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder, characterized by the presence of eosinophilic inclusions (NIIs) within nuclei of central and peripheral nervous system cells. This study aims to identify the components of NIIs, which have been difficult to analyze directly due to their insolubility In order to establish a method to directly identify the components of NIIs, we first analyzed the huntingtin inclusion-rich fraction obtained from the brains of Huntington disease model mice. Although the sequence with expanded polyglutamine could not be identified by liquid-chromatog. mass spectrometry, amino acid anal. revealed that glutamine of the huntingtin inclusion-rich fraction increased significantly. This is compatible with the calculated amino acid content of the transgene product. Therefore, we applied this method to analyze the NIIs of diseased human brains, which may have proteins with compositionally biased regions, and identified a serine-rich protein called hornerin. Since the analyzed NII-rich fraction was also serine-rich, we suggested hornerin as a major component of the NIIs. A specific distribution of hornerin in NIID was also investigated by Matrix-assisted laser desorption/ionization imaging mass spectrometry and immunofluorescence. Finally, we confirmed a variant of hornerin by whole-exome sequencing and DNA sequencing. This study suggests that hornerin may be related to the pathol. process of this NIID, and the direct anal. of NIIs, especially by amino acid anal. using the NII-rich fractions, would contribute to a deeper understanding of the disease pathogenesis.
Acta Neuropathologica Communications published new progress about 63-68-3. 63-68-3 belongs to catalysis-chemistry, auxiliary class Natural product, name is (S)-2-Amino-4-(methylthio)butanoic acid, and the molecular formula is C5H11NO2S, SDS of cas: 63-68-3.
Referemce:
https://courses.lumenlearning.com/boundless-chemistry/chapter/catalysis/,
Catalysis – Wikipedia