Abstract：
:Prions are proteins that access self-templating amyloid forms, which confer phenotypic changes that can spread from individual to individual within or between species. These infectious phenotypes can be beneficial, as with yeast prions, or deleterious, as with mammalian prions that transmit spongiform encephalopathies. However, the ability to form self-templating amyloid is not unique to prion proteins. Diverse polypeptides that tend to populate intrinsically unfolded states also form self-templating amyloid conformers that are associated with devastating neurodegenerative disorders. Moreover, two RNA-binding proteins, FUS and TDP-43, which form cytoplasmic aggregates in amyotrophic lateral sclerosis, harbor a 'prion domain' similar to those found in several yeast prion proteins. Can these proteins and the neurodegenerative diseases to which they are linked become 'infectious' too? Here, we highlight advances that define the transmissibility of amyloid forms connected with Alzheimer's disease, Parkinson's disease and Huntington's disease. Collectively, these findings suggest that amyloid conformers can spread from cell to cell within the brains of afflicted individuals, thereby spreading the specific neurodegenerative phenotypes distinctive to the protein being converted to amyloid. Importantly, this transmissibility mandates a re-evaluation of emerging neuronal graft and stem-cell therapies. In this Commentary, we suggest how these treatments might be optimized to overcome the transmissible conformers that confer neurodegeneration.
journal_name
J Cell Scijournal_title
Journal of cell scienceauthors
Cushman M,Johnson BS,King OD,Gitler AD,Shorter Jdoi
10.1242/jcs.051672subject
Has Abstractpub_date
2010-04-15 00:00:00pages
1191-201issue
Pt 8eissn
0021-9533issn
1477-9137pii
123/8/1191journal_volume
123pub_type
杂志文章abstract：:Nearly all intermediate filament (IF) proteins share two sequence motifs located at the N- and the C-terminal ends of their helical rod domain ('coil 1a' and 'coil 2b', respectively). To examine the structural role of the coil 2b motif, we have performed in vitro assembly studies and in vivo microinjection experiments...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1992-05-01 00:00:00
abstract：:Plasmid DNA or artificial mRNA injected intramuscularly into skeletal muscle via a 27 g needle expressed transgenes at relatively efficient levels in skeletal myofibers and cardiac cells. In the present study, several approaches were used to determine the mechanism of cellular uptake. After exposure of naked plasmid D...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1992-12-01 00:00:00
abstract：:We have previously shown that Xenopus oocytes, eggs, and early embryos contain lamins LII and LIII, and that portions of each are associated with distinct egg vesicle populations. We now report that a lamin similar or identical to the B-type lamin LI is also present in oocyte nuclei and in egg extracts. We have quanti...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1996-07-01 00:00:00
abstract：:The translationally controlled tumor protein (TCTP; also known as TPT1 in mammals) is highly conserved and ubiquitously expressed in eukaryotes. It is involved in growth and development, cell cycle progression, protection against cellular stresses and apoptosis, indicating the multifunctional role of the protein. Here...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.206417
更新日期：2018-05-10 00:00:00
abstract：:Fatty acids constitute an important energy source for various tissues. The mechanisms that mediate and control uptake of free fatty acids from the circulation, however, are poorly understood. Here we show that efficient fatty-acid uptake by yeast cells requires the protein kinase Ypk1, the orthologue of the human seru...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.063073
更新日期：2010-07-01 00:00:00
abstract：:Actin polymerization is a crucial process during sperm capacitation. We have recently described the participation of FAK during actin polymerization in guinea pig spermatozoa. However, the mechanism by which FAK mediates these processes is unknown. Our previous data have shown that MAPK1 (hereafter referred to as ERK2...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.239186
更新日期：2020-04-28 00:00:00
abstract：:The translationally controlled protein P23 was discovered by the early induction of its rate of synthesis after mitogenic stimulation of mouse fibroblasts. P23 is expressed in almost all mammalian tissues and it is highly conserved between animals, plants and yeast. Based on its amino acid sequence, P23 cannot be attr...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1999-04-01 00:00:00
abstract：:Two isoforms of laminin were extracted from human placenta by neutral buffer containing EDTA, copurified through several steps and finally separated by Mono Q anion exchange chromatography. One variant consisted of disulphide-linked 340, 230 and 190 kDa subunits, which were identified by immunoblotting as Am, B1e and ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1994-01-01 00:00:00
abstract：:Entry into mitosis requires p34(cdc2), which activates downstream mitotic events through phosphorylation of key target proteins. In Aspergillus nidulans, the NIMA protein kinase has been identified as a potential downstream target and plays a role in regulating chromatin condensation at mitosis. nimA- mutants arrest i...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1998-04-01 00:00:00
abstract：:We first identified Bves (blood vessel/epicardial substance) as a transmembrane protein that localized to the lateral compartment of the epithelial epicardium. Bves traffics to sites of cell-cell contact in cultured epicardial cells and promotes adhesion following transfection into non-adherent fibroblastic L-cells, r...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.02588
更新日期：2005-10-15 00:00:00
abstract：:Phosphatidylinositol phosphates are important regulators of processes such as the cytoskeleton organization, membrane trafficking and gene transcription, which are all crucial for polarized cell growth. In particular, phosphatidylinositol 4,5-bisphosphate [PtdIns(4,5)P2] has essential roles in polarized growth as well...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.122606
更新日期：2013-08-15 00:00:00
abstract：:The contractile vacuole complex (CVC) of Trypanosoma cruzi, the etiologic agent of Chagas disease, collects and expels excess water as a mechanism of regulatory volume decrease after hyposmotic stress; it also has a role in cell shrinking after hyperosmotic stress. Here, we report that, in addition to its role in osmo...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.169466
更新日期：2015-06-15 00:00:00
abstract：:Protein traffic moving from the endoplasmic reticulum (ER) to the Golgi complex in mammalian cells passes through the tubulovesicular membrane clusters of the ER-Golgi intermediate compartment (ERGIC), the marker of which is the lectin ERGIC-53. The dynamic nature and functional role of the ERGIC have been debated for...
journal_title：Journal of cell science
pub_type： 杂志文章,评审
doi：10.1242/jcs.03019
更新日期：2006-06-01 00:00:00
abstract：:Actin assembly is involved in cell motility and intracellular movement of Listeria monocytogenes. Induction of Listeria actin tails is mediated by the surface protein ActA. The N-terminal domain of ActA is sufficient for this function. Cell components known to play a role in the actin-based motility of Listeria are VA...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1998-10-01 00:00:00
abstract：:Using a genome-wide technical knockout, we isolated a newly identified set of GRIM (genes associated with retinoid-interferon-induced mortality) genes; GRIM genes mediate IFN- and retinoic-acid (RA)-induced cell death. Here, we describe the isolation and characterization of one such gene, GRIM-1. Three proteins, with ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.070250
更新日期：2010-08-15 00:00:00
abstract：:Increases in cytosolic Ca(2+) concentration ([Ca(2+)](c)) mediated by inositol (1,4,5)-trisphosphate [Ins(1,4,5)P(3), hereafter InsP(3)] regulate activities that include division, contraction and cell death. InsP(3)-evoked Ca(2+) release often begins at a single site, then regeneratively propagates through the cell as...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.105163
更新日期：2012-11-15 00:00:00
abstract：:Human procathepsin D carries two N-linked glycosylation sites at asparagine residues 70 and 199, widely separated on the surface of the folded protein. We created monoglycosylated procathepsin D molecules by site-directed mutagenesis in vitro of the individual glycosylation sites. With only two exceptions, all 12 muta...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1995-05-01 00:00:00
abstract：:Mammalian homologs of the Drosophila TRP protein have been shown to form cation-permeable channels in the plasma membrane but very little is known about the mechanisms that control their cell surface localization. Recently it has been demonstrated that the last three C-terminal amino acids (TRL) of TRPC4 comprise a PD...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2002-09-01 00:00:00
abstract：:Axonemal precursor tubulin is the major protein component of the detergent-soluble membrane/matrix fraction of sea urchin embryonic cilia. Its unusual abundance may reflect the rapid turnover of these cilia, a process that is further documented here. However, whether during induced regeneration or normal turnover and ...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1994-02-01 00:00:00
abstract：:The protein kinase inhibitor H-7 prevents the assembly of tight junctions in cultured Madin Darby Canine Kidney (MDCK) epithelial cells (Balda et al. (1991) J. Membr. Biol. 122, 193-202; Nigam et al. (1991) Biochem. Biophys. Res. Commun. 181, 548-553); however, its mechanism of action is unknown. To understand the bas...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1994-04-01 00:00:00
abstract：:Cytokines, such as interferons, erythropoietin, leptin and most interleukins, signal through type 1 cytokine receptors and activate the canonical JAK-STAT pathway. Aberrant cytokine signalling underlies numerous pathologies and adequate, temporary receptor activation is therefore under tight control. Negative-feedback...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.078055
更新日期：2011-03-15 00:00:00
abstract：:Meiosis is characterized by two chromosome segregation rounds (meiosis I and II), which follow a single round of DNA replication, resulting in haploid genome formation. Chromosome reduction occurs at meiosis I. It relies on key structures, such as chiasmata, which are formed by repair of double-strand breaks (DSBs) be...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.152520
更新日期：2014-11-01 00:00:00
abstract：:Rubella virus (RV) envelope glycoproteins E1 and E2 are targeted to the Golgi as heterodimers. While E2 contains a transmembrane Golgi retention signal, E1 is arrested in a pre-Golgi compartment in the absence of E2, and appears to require heterodimerization in order to reach the Golgi. Various forms of E1 with deleti...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1996-06-01 00:00:00
abstract：:Tissues fixed in osmium tetroxide or in combined osmium and glutaraldehyde (Hinde), embedded in Spurr's medium, cut at 0-5-I mum and mounted in Farrants' gum medium containing ethyl gallate, show good staining of lipid-contaning structures (droplets of triglyceride, membranes, mitochondria, etc.) in the light microsco...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1975-12-01 00:00:00
abstract：:Many pathways dysregulated in prostate cancer are also involved in epithelial differentiation. To better understand prostate tumor initiation, we sought to investigate specific genes and mechanisms required for normal basal to luminal cell differentiation. Utilizing human prostate basal epithelial cells and an in vitr...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.197152
更新日期：2017-06-01 00:00:00
abstract：:As previously described, a cell surface-associated adhesive factor (AF) was separated from differentiated rat ascites hepatoma AH136B cells (forming cell islands in vivo) and highly purified by chromatography. AF synthesis by the cells was investigated by means of ultrastructural immunoperoxidase cytochemistry. Its sy...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1984-10-01 00:00:00
abstract：:Elementary Ca(2+) signals, such as "Ca(2+) puffs", which arise from the activation of inositol 1,4,5-trisphosphate receptors, are building blocks for local and global Ca(2+) signalling. We characterized Ca(2+) puffs in six cell types that expressed differing ratios of the three inositol 1,4,5-trisphosphate receptor is...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：2001-11-01 00:00:00
abstract：:Previously we cloned and characterized the hamster intermediate filament genes coding for vimentin and desmin. It was demonstrated that the cloned desmin gene was expressed after gene transfer and that the newly synthesized protein assembles into intermediate filaments. Here we present data on the transfection of modi...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：
更新日期：1987-11-01 00:00:00
abstract：:Nemaline myopathy (NM) is a congenital myopathy with an estimated incidence of 150,000 live births. It is caused by mutations in thin filament components, including nebulin, which accounts for about 50% of the cases. The identification of NM cases with nonsense mutations resulting in loss of the extreme C-terminal SH3...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.137026
更新日期：2013-12-01 00:00:00
abstract：:Cyclin G (CycG) belongs to the atypical cyclins, which have diverse cellular functions. The two mammalian CycG genes, CycG1 and CycG2, regulate the cell cycle in response to cell stress. Detailed analyses of the role of the single Drosophila cycG gene have been hampered by the lack of a mutant. We generated a null mut...
journal_title：Journal of cell science
pub_type： 杂志文章
doi：10.1242/jcs.113902
更新日期：2012-11-15 00:00:00
