Glial S100B Positive Vacuoles In Purkinje Cells: Earliest Morphological Abnormality In SCA1 Transgenic Mice
Journal Title: Journal of Neurological Sciences-Turkish - Year 2006, Vol 23, Issue 3
Abstract
Spinocerebellar ataxia-1 (SCA1) is caused by the expansion of a polyglutamine repeat within the disease protein, ataxin-1. The overexpression of mutant ataxin-1 in SCA1 transgenic mice results in the formation of cytoplasmic vacuoles in Purkinje neurons (PKN) of the cerebellum. PKN are closely associated with neighboring Bergmann glia. To elucidate the role of Bergmann glia in SCA1 pathogenesis, cerebellar tissue from 7 days to 6 wks old SCA1 transgenic and wildtype mice were used. We observed that Bergmann glial S100B protein is localized to the cytoplasmic vacuoles in SCA1 PKN. These S100B positive cytoplasmic vacuoles began appearing much before the onset of behavioral abnormalities, and were negative for other glial and PKN marker proteins. Electron micrographs revealed that vacuoles have a double membrane. In the vacuoles, S100B colocalized with receptors of advanced glycation end-products (RAGE), and S100B co-immunoprecipated with cerebellar RAGE. In SCA1 PKN cultures, exogenous S100B protein interacted with the PKN membranes and was internalized. These data suggest that glial S100B though extrinsic to PKN is sequestered into cytoplasmic vacuoles in SCA1 mice at early postnatal ages. Further, S100B may be binding to RAGE on Purkinje cell membranes before these membranes are internalized.
Authors and Affiliations
Parminder VIG, Maripar LOPEZ, Jinrong WEI, S SUBRAMONY, Jeffrey HENEGAR, Jonathan FRATKIN
Keywords
Related Articles
- EP ID EP83703
- DOI -
- Views 100
- Downloads 0
The Effects of Female Sexual Hormones and Estrogen Receptor Agonists on The NF-κB p50 and p65 Levels Following TBI in Rat Intestine
Objectives: The purpose of this study was to evaluate the role of female sexual hormones on the intestinal NF-κB levels following traumatic brain injury (TBI) induction. Materials and methods: Diffuse TBI was induced by...
Granülosit Makrofaj Koloni-Stimüle Edici Faktör ile Uyarılmış Mikrogliya, Deneysel Otoimmun Ensefalomiyelitin Başlamasına ve Sürdürülmesine Katkıda Bulunur
Deneysel otoimmun ensefalomiyelit (EAE) modelinde merkezi sinir sistemindeki (MSS) T hücrelerinin yok edilmesi ve yayılımı iyi anlaşılamamış olmasına rağmen, dokuya yayılmış olan immun hücreler ile bölgesel gliyal hücrel...
Low Molecular Weight Heparin Induced Delayed Traumatic Clival Subdural Hematoma Associated With Isolated Abducens Nerve Palsy in a Child
Traumatic clival subdural hematoma is a very rare clinical entity. This rarity can result in delayed or missed diagnoses. Although several hypotheses are proposed to explain this lesion, the exact pathophysiology remains...
Ataxic gait due to Giant Cystic Virchow-Robin Spaces: Case Report
Virchow-Robin spaces (VRSs) are perivascular pia-lined extensions of the subarachnoid space around the arteries and veins as they enter the brain parenchyma. The precise etiology of these enlarged perivascular spaces (PV...
Exteroceptive Suppression of Masseter In Behçet’s Disease
Objectives: Brainstem is the most frequently affected neural substrate in neurological involvement due to Behçet’s disease (neuro-Behçet). The demonstration of brainstem involvement in Behçet’s disease (BD) is important...