title: G protein-coupled receptor 26 immunoreactivity in intranuclear inclusions associated with polyglutamine and intranuclear inclusion body diseases.
abstract: G protein-coupled receptor 26 (GPR26) is one of the G-protein-coupled receptors (GPCRs), which comprise the largest family of membrane proteins and mediate most of the physiological responses to hormones, neurotransmitters and environmental stimulants. Although GPCRs are considered to play an important role in the pathophysiology of neurodegenerative disorders, it is uncertain whether GPR26 is involved in the pathogenesis of polyglutamine and intranuclear inclusion body diseases. We immunohistochemically examined the brain tissues of patients with four polyglutamine diseases (Huntington's disease, dentatorubral-pallidoluysian atrophy, and spinocerebellar ataxia types 1 and 3) and intranuclear inclusion body disease, and normal control subjects. In controls, anti-GPR26 antibody immunolabeled the neuronal cytoplasm in a diffuse granular pattern. Neuronal nuclear inclusions in polyglutamine diseases were immunopositive for GPR26. In intranuclear inclusion body disease, GPR26-positive nuclear inclusions were found in both neurons and glial cells. Marinesco bodies in aged control subjects were also positive for GPR26. Double immunofluorescence analysis revealed co-localization of GPR26 with polyglutamine or ubiquitin in these nuclear inclusions. These findings suggest that GPR26 may have a common role in the formation or degradation of intranuclear inclusions in several neurodegenerative diseases.
authors: Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki.
authors: Fumiaki
authors: F
authors: Mori
authors: Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki.
authors: Kunikazu
authors: K
authors: Tanji
authors: Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki.
authors: Yasuo
authors: Y
authors: Miki
authors: Department of Pathology, Brain Research Institute, University of Niigata, Niigata.
authors: Yasuko
authors: Y
authors: Toyoshima
authors: Department of Neuropathology, Aichi Medical University, Nagakute.
authors: Mari
authors: M
authors: Yoshida
authors: Department of Pathological Neuroscience, Center for Bioresource-based Researches, Brain Research Institute, University of Niigata, Niigata.
authors: Akiyoshi
authors: A
authors: Kakita
authors: Department of Pathology, Brain Research Institute, University of Niigata, Niigata.
authors: Hitoshi
authors: H
authors: Takahashi
authors: Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors: Jun
authors: J
authors: Utsumi
authors: Department of Neurology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors: Hidenao
authors: H
authors: Sasaki
authors: Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki.
authors: Koichi
authors: K
authors: Wakabayashi
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001344:Autopsy; D001921:Brain / Q000473:pathology; D005260:Female; D020271:Heredodegenerative Disorders, Nervous System / Q000235:genetics / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D045586:Intranuclear Inclusion Bodies / Q000235:genetics* / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000473:pathology; D010455:Peptides / Q000235:genetics* / Q000378:metabolism*; D043562:Receptors, G-Protein-Coupled / Q000235:genetics* / Q000378:metabolism*; D025801:Ubiquitin / Q000235:genetics / Q000378:metabolism
chemical_list: C489011:GPR26 protein, human; D010455:Peptides; D043562:Receptors, G-Protein-Coupled; D025801:Ubiquitin; C097188:polyglutamine
keywords: G protein-coupled receptor 26; Marinesco body; immunohistochemistry; intranuclear inclusion body disease; polyglutamine disease