PMID- 10454141 OWN - NLM STAT- MEDLINE DCOM- 19990916 LR - 20190701 IS - 0304-3940 (Print) IS - 0304-3940 (Linking) VI - 270 IP - 1 DP - 1999 Jul 23 TI - Expression of wild-type and V210I mutant prion protein in human neuroblastoma cells. PG - 41-4 AB - The conversion of the host-encoded prion protein (PrPc) into the insoluble, protease-resistant isoform (PrPsc) is the main pathogenic mechanism of transmissible spongiform encephalopathies. They are fatal neurodegenerative disorders, which in human occur as sporadic, inherited or familial forms. These last forms are linked to insert or point mutations of PrPc which may facilitate the spontaneous conversion into PrPsc. We have established stably transfected human neuroblastoma cells (SH-SY5Y) expressing mutant V210I, or wild-type PrPc. Both proteins were expressed and attached to the cell surface. The mutation in position 210 did not alter the biochemical properties of the protein in comparison with the wild-type protein nor induced any conformational changes similar to those observed in PrPsc. FAU - Vetrugno, V AU - Vetrugno V AD - Laboratory of Virology, Istituto Superiore di Sanita, Rome, Italy. FAU - Malchow, M AU - Malchow M FAU - Liu, Q AU - Liu Q FAU - Marziali, G AU - Marziali G FAU - Battistini, A AU - Battistini A FAU - Pocchiari, M AU - Pocchiari M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - Ireland TA - Neurosci Lett JT - Neuroscience letters JID - 7600130 RN - 0 (Amyloid) RN - 0 (PRNP protein, human) RN - 0 (PrPC Proteins) RN - 0 (PrPSc Proteins) RN - 0 (Prion Proteins) RN - 0 (Prions) RN - 0 (Protein Precursors) RN - 0 (Recombinant Proteins) RN - EC 3.1.4.- (Type C Phospholipases) RN - EC 4.6.1.13 (Phosphatidylinositol Diacylglycerol-Lyase) SB - IM MH - Amino Acid Substitution MH - Amyloid/biosynthesis/genetics MH - Brain Neoplasms MH - Cell Membrane/metabolism MH - Humans MH - Neuroblastoma MH - Open Reading Frames MH - Phosphatidylinositol Diacylglycerol-Lyase MH - Point Mutation MH - PrPC Proteins/biosynthesis/*genetics MH - PrPSc Proteins/biosynthesis/*genetics MH - Prion Proteins MH - Prions MH - Protein Precursors/biosynthesis/genetics MH - Recombinant Proteins/biosynthesis MH - Transfection MH - Tumor Cells, Cultured MH - Type C Phospholipases/metabolism EDAT- 1999/08/24 00:00 MHDA- 1999/08/24 00:01 CRDT- 1999/08/24 00:00 PHST- 1999/08/24 00:00 [pubmed] PHST- 1999/08/24 00:01 [medline] PHST- 1999/08/24 00:00 [entrez] AID - S0304394099004607 [pii] AID - 10.1016/s0304-3940(99)00460-7 [doi] PST - ppublish SO - Neurosci Lett. 1999 Jul 23;270(1):41-4. doi: 10.1016/s0304-3940(99)00460-7.