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Immunohistochemical localization of exoribonucleases (DIS3L2 and XRN1) in intranuclear inclusion body disease
- Source :
- Neuroscience letters. 662
- Publication Year :
- 2017
-
Abstract
- mRNA turnover controls gene expression under various conditions in aging and neurodegenerative diseases. Polyglutamine (polyQ) diseases and intranuclear inclusion body disease (INIBD) are neurodegenerative diseases characterized by the formation of nuclear inclusions. These inclusions are immunopositive for several proteins associated with amyotrophic lateral sclerosis. In amyotrophic lateral sclerosis, the processing of RNA from gene transcription through degradation (RNA metabolism) has been reported to be altered. We hypothesized that the proteins associated with RNA metabolism are also involved in the formation of nuclear inclusions in polyQ diseases and INIBD. 3'-5' exoribonuclease DIS3L2 and 5'-3' exoribonuclease XRN1 play critical roles in mRNA decay. Using immunohistochemistry with antibodies against DIS3L2 and XRN1, we examined the brains of patients with polyQ diseases and INIBD and normal control subjects. In controls, immunoreactivity for DIS3L2 and XRN1 was found in the neuronal cytoplasm and neuropil, respectively. In INIBD, immunoreactivity for DIS3L2 and XRN1 was present in neuronal and glial nuclear inclusions. Co-localization of ubiquitin and DIS3L2 or XRN1 was demonstrated in these inclusions. In polyQ diseases, however, nuclear inclusions were immunonegative for DIS3L2 and XRN1. These findings suggest that sequestration of exoribonucleases to nuclear inclusions may be related to the pathogenesis of INIBD.
- Subjects :
- 0301 basic medicine
Male
Pathology
medicine.medical_specialty
Intranuclear Inclusion Bodies
Biology
03 medical and health sciences
0302 clinical medicine
Ubiquitin
Exoribonuclease
Gene expression
Neuropil
medicine
Humans
Amyotrophic lateral sclerosis
Aged
Aged, 80 and over
Messenger RNA
General Neuroscience
RNA
Brain
Neurodegenerative Diseases
Middle Aged
medicine.disease
Immunohistochemistry
Cell biology
030104 developmental biology
medicine.anatomical_structure
Cytoplasm
Exoribonucleases
biology.protein
Female
Microtubule-Associated Proteins
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 18727972
- Volume :
- 662
- Database :
- OpenAIRE
- Journal :
- Neuroscience letters
- Accession number :
- edsair.doi.dedup.....c3f0665dd477042e45b53aa71e6f0750