Exocytosis is impaired in mucopolysaccharidosis IIIA mouse chromaffin cells

• Published in: Neuroscience Vol. 227; pp. 110 - 118
• Main Authors: Keating, D.J, Winter, M.A, Hemsley, K.M, Mackenzie, K.D, Teo, E.H, Hopwood, J.J, Brooks, D.A, Parkinson-Lawrence, E.J
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Ltd 27.12.2012; Elsevier
• Subjects: Adrenal Glands - metabolism; Adrenal Glands - pathology; Adrenal Glands - ultrastructure; Analysis of Variance; Animals; Biological and medical sciences; Calcium - metabolism; Carbohydrates (enzymatic deficiencies). Glycogenosis; Carbon; carbon fibre amperometry; Catecholamines - metabolism; Cells, Cultured; chromaffin cells; Chromaffin Cells - physiology; Chromaffin Cells - ultrastructure; Disease Models, Animal; Errors of metabolism; exocytosis; Exocytosis - genetics; Heparitin Sulfate - metabolism; Lysosomes - metabolism; Lysosomes - pathology; Medical sciences; Metabolic diseases; Mice; Mice, Inbred C57BL; Mice, Transgenic; Microscopy, Electron, Transmission; mouse; Mucopolysaccharidosis III - genetics; Mucopolysaccharidosis III - pathology; mucopolysaccharidosis IIIA; Neurology; neurotransmission; Statistics, Nonparametric; heparan sulphate-derived disaccharide glucosamine N-sulphate [α-1,4] uronic acid; 4-(2-hydroxyethyl)-1-piperazineethanesulfonic acid; PSF; GlcNS-UA; neurotransmission; dimethylformamide; Hepes; root mean squared; DMEM; mouse; chromaffin cells; CYWHS; Children Youth and Women’s Health Service; pre-spike “foot”; Dulbecco’s modified Eagle medium; RMS; carbon fibre amperometry; exocytosis; mucopolysaccharidosis IIIA; DMF; MPS IIIA; Endocrine gland; Adrenal medulla; Diseases of the osteoarticular system; Rodentia; Metabolic diseases; Mucopolysaccharidosis; Chromaffin cell; Exocytosis; Enzymopathy; Genetic disease; Vertebrata; Mammalia; Mouse; Animal; Carbohydrate; Neurotransmission
• ISSN: 0306-4522; 1873-7544
• DOI: 10.1016/j.neuroscience.2012.09.034

Highlights ► Elevated heparan sulphate storage in MPS IIIA chromaffin cells. ► Catecholamine secretion was reduced in MPS IIIA chromaffin cells. ► Fusion pore kinetics was unaltered in MPS IIIA chromaffin cells. ► Calcium entry was unaltered in MPS IIIA chromaffin cells.