Altered olfactory epithelial structure and function in feline models of mucopolysaccharidoses I and VI
| Autor: | Luba Dankulich-Nagrudny, Fritz W. Lischka, Nancy E. Rawson, Karen K. Yee, George Gomez, Leen Lo, Mark E. Haskins |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Male
Cell physiology congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Pathology Patch-Clamp Techniques Mucopolysaccharidosis I Central nervous system Biology Article Olfactory Receptor Neurons Glycosaminoglycan Olfactory mucosa Calcium imaging Olfactory Mucosa Internal medicine medicine Animals Humans skin and connective tissue diseases Cells Cultured Mucopolysaccharidosis VI Olfactory receptor CATS General Neuroscience nutritional and metabolic diseases Disease Models Animal Endocrinology medicine.anatomical_structure Cats Female Nasal Cavity |
| Zdroj: | The Journal of Comparative Neurology. 511:360-372 |
| ISSN: | 1096-9861 0021-9967 |
| DOI: | 10.1002/cne.21847 |
| Popis: | The mucopolysaccharidoses (MPS) are a family of lysosomal storage diseases resulting in developmental defects and, in some types, mental retardation and other neurological symptoms. To gain insight into the neurological dysfunction in MPS, we examined the morphology of olfactory epithelia (OE) and physiology of olfactory receptor neurons (ORNs) in cat models of MPS I, a type in which neuronal lesions are prominent, and MPS VI, in which they are essentially absent. Histopathology showed that both groups of MPS-affected cats had significantly thinner olfactory epithelia than controls. While immature and mature ORNs were present in both MPS I and VI affected OE, the OE of MPS I-affected cats was structurally disorganized. ORN function was assessed with calcium imaging and patch-clamp recording. Few viable ORNs were recovered from MPS VI cats, but these exhibited normal responses to odors and pharmacological stimuli. In contrast, viable ORNs were as prevalent in MPS I as in controls, but were significantly less likely to respond to odor stimuli, although other responses were normal. Disrupted OE organization and impaired ORN function in MPS I, but not MPS VI, corresponds to the central nervous system (CNS) lesions found in MPS I but not MPS VI. These data represent the first neurophysiological correlate of this correspondence and have implications for understanding both the role of glycosaminoglycans in maintenance of the OE, as well as for targeting further research into the basis for and treatment of the neurological consequences of MPS disorders. |
| Databáze: | OpenAIRE |
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