Introduction: The receptor for advanced glycation end-products (RAGE) is a multi-ligand receptor that belongs to the immunoglobulin superfamily of cell surface receptors, whose ligands are known to be upregulated in neuropathological conditions. RAGE up-regulation has been described in neurodegenerative diseases, such as Alzheimer’s disease, Creutzfeldt-Jakob disease and Huntington’s disease (HD) [1]. Materials and methods: To analyze in detail the implication of RAGE in HD, we studied the immunohistochemical distribution of RAGE in the striatum of the R6/2 mouse model of HD, with particular attention to the neuronal subpopulations and their relative vulnerability to HD neurodegeneration. Results: We show that RAGE immunoreactivity munoreactivity is evenly distributed to the cytoplasm of neurons in the wild type mouse, while it is spot-like in the R6/2 mouse. Moreover, RAGE is expressed in the striatum with an uneven distribution that reminds of the striosome pattern, but does not overlap with the calbindin-labeled patch-matrix compartmentalization. RAGE is distributed in 90% of spiny projection neurons, both in the normal mouse and in the R6/2. RAGE co-localizes with all of the striatal interneuron subsets both in the wild-type and in the R6/2 mouse. However, the intensity ofRAGEimmunoreactivity is significantly higher in the spiny neurons and in the PARV and CALR neurons of R6/2 mouse, whereas it is comparable between R6/2 and wild-type in the cholinergic and somatostatinergic interneurons. Conclusions: These data support the concept that RAGE is upregulated in the neurodegenerative process of HD, and suggests that its activation is related to the individual vulnerability of the striatal neuronal subtype. References [1] Ma et al. 2004.
Immunohistochemical localization of Receptor for advanced glycation end products (RAGE) in the R6/2 mouse model of Huntington’s disease
MELONE, Mariarosa Anna Beatrice;
2010
Abstract
Introduction: The receptor for advanced glycation end-products (RAGE) is a multi-ligand receptor that belongs to the immunoglobulin superfamily of cell surface receptors, whose ligands are known to be upregulated in neuropathological conditions. RAGE up-regulation has been described in neurodegenerative diseases, such as Alzheimer’s disease, Creutzfeldt-Jakob disease and Huntington’s disease (HD) [1]. Materials and methods: To analyze in detail the implication of RAGE in HD, we studied the immunohistochemical distribution of RAGE in the striatum of the R6/2 mouse model of HD, with particular attention to the neuronal subpopulations and their relative vulnerability to HD neurodegeneration. Results: We show that RAGE immunoreactivity munoreactivity is evenly distributed to the cytoplasm of neurons in the wild type mouse, while it is spot-like in the R6/2 mouse. Moreover, RAGE is expressed in the striatum with an uneven distribution that reminds of the striosome pattern, but does not overlap with the calbindin-labeled patch-matrix compartmentalization. RAGE is distributed in 90% of spiny projection neurons, both in the normal mouse and in the R6/2. RAGE co-localizes with all of the striatal interneuron subsets both in the wild-type and in the R6/2 mouse. However, the intensity ofRAGEimmunoreactivity is significantly higher in the spiny neurons and in the PARV and CALR neurons of R6/2 mouse, whereas it is comparable between R6/2 and wild-type in the cholinergic and somatostatinergic interneurons. Conclusions: These data support the concept that RAGE is upregulated in the neurodegenerative process of HD, and suggests that its activation is related to the individual vulnerability of the striatal neuronal subtype. References [1] Ma et al. 2004.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
