Normal Development of Nerve--Muscle Synapses in Mice Lacking the Prion Protein Gene

• Published in: Proceedings of the Royal Society. B, Biological sciences Vol. 250; no. 1328; pp. 151 - 155
• Main Authors: Brenner, H. R., Herczeg, A., Oesch, Bruno
• Format: Journal Article
• Language: English
• Published: London The Royal Society 23-11-1992; Royal Society of London
• Subjects: Animals; Arias; Biological and medical sciences; Blotting, Northern; Cell receptors; Cell structures and functions; Cholinergic receptors; Electrophysiology; Fundamental and applied biological sciences. Psychology; Gene expression regulation; In situ hybridization; Macromolecular Substances; Messenger RNA; Mice; Mice, Mutant Strains; Molecular and cellular biology; Monoamines receptors (catecholamine, serotonine, histamine, acetylcholine); Muscle Denervation; Muscle fibers; Muscles; Muscles - physiology; Neuromuscular Junction - physiology; Neurons; Prions; Prions - genetics; PrPSc Proteins; Receptors, Cholinergic - genetics; Receptors, Cholinergic - physiology; RNA - genetics; RNA - isolation & purification; RNA, Messenger - metabolism; Space life sciences; Spinal Cord - physiology; Synapses; Synapses - physiology; Vertebrata; Cholinergic receptor; Synapse; Mammalia; Mouse; Rodentia; Trophic factor; Prion; Gene expression; Striated muscle; Neuromuscular junction
• ISSN: 0962-8452; 1471-2954
• DOI: 10.1098/rspb.1992.0143

The expression of acetylcholine receptors (AChR) at neuromuscular synapses in skeletal muscle is regulated by innervation. Recent evidence suggests that the neurotrophic factors involved in the expression of AChR subunit genes may be related to the prion protein (PrPc), a protein of unknown function expressed primarily in neurons which, in its modified form, PrPSc, is thought to have a role in the pathogenesis of transmissible spongiform encephalopathies. We have tested for an involvement of PrPc in the neurotrophic regulation of synaptic AChRs in muscle by comparing the contents of AChR ε- and γ-subunit mRNAs by Northern blot analysis and by in situ hybridization in mice with normal and with deleted PrP genes. At the protein level, AChR expression was assessed electrophysiologically. No difference was found between muscles from the two types of animals, suggesting that the neural regulation of AChR subunit expression in skeletal muscle can be mediated by factors that are not derived from the PrP gene.