The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents.

Abstract:

:Human sporadic Creutzfeldt-Jakob disease (sCJD), endemic sheep scrapie, and epidemic bovine spongiform encephalopathy (BSE) are caused by a related group of infectious agents. The new U.K. BSE agent spread to many species, including humans, and clarifying the origin, specificity, virulence, and diversity of these agents is critical, particularly because infected humans do not develop disease for many years. As with viruses, transmissible spongiform encephalopathy (TSE) agents can adapt to new species and become more virulent yet maintain fundamentally unique and stable identities. To make agent differences manifest, one must keep the host genotype constant. Many TSE agents have revealed their independent identities in normal mice. We transmitted primate kuru, a TSE once epidemic in New Guinea, to mice expressing normal and approximately 8-fold higher levels of murine prion protein (PrP). High levels of murine PrP did not prevent infection but instead shortened incubation time, as would be expected for a viral receptor. Sporadic CJD and BSE agents and representative scrapie agents were clearly different from kuru in incubation time, brain neuropathology, and lymphoreticular involvement. Many TSE agents can infect monotypic cultured GT1 cells, and unlike sporadic CJD isolates, kuru rapidly and stably infected these cells. The geographic independence of the kuru agent provides additional reasons to explore causal environmental pathogens in these infectious neurodegenerative diseases.

authors

Manuelidis L,Chakrabarty T,Miyazawa K,Nduom NA,Emmerling K

doi

10.1073/pnas.0905825106

subject

Has Abstract

pub_date

2009-08-11 00:00:00

pages

13529-34

issue

32

eissn

0027-8424

issn

1091-6490

pii

0905825106

journal_volume

106

pub_type

杂志文章
  • NF-kappaB activation and potentiation of proinflammatory responses by the Helicobacter pylori CagA protein.

    abstract::The Helicobacter pylori immunodominant protein, CagA, is associated with severe gastritis and carcinoma. Injection of CagA into gastric epithelial cells by type IV secretion leads to actin-cytoskeletal rearrangements and cell scattering. CagA has been reported to have no role in the induction of transcription factor N...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0409873102

    authors: Brandt S,Kwok T,Hartig R,König W,Backert S

    更新日期:2005-06-28 00:00:00

  • Regulation of the production of secretory proteins: intracellular degradation of newly synthesized "defective" collagen.

    abstract::Confluent cultures of human fetal lung fibroblasts degrade approximately 10% of their newly synthesized collagen within the cell prior to secretion. This basal level of intracellular degradation could not be inhibited by colchicine or cytochalasin B, inhibitors of microtubular and microfilament function, respectively,...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.77.8.4746

    authors: Berg RA,Schwartz ML,Crystal RG

    更新日期:1980-08-01 00:00:00

  • Kras mutant genetically engineered mouse models of human cancers are genomically heterogeneous.

    abstract::KRAS mutant tumors are largely recalcitrant to targeted therapies. Genetically engineered mouse models (GEMMs) of Kras mutant cancer recapitulate critical aspects of this disease and are widely used for preclinical validation of targets and therapies. Through comprehensive profiling of exomes and matched transcriptome...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1708391114

    authors: Chung WJ,Daemen A,Cheng JH,Long JE,Cooper JE,Wang BE,Tran C,Singh M,Gnad F,Modrusan Z,Foreman O,Junttila MR

    更新日期:2017-12-19 00:00:00

  • Sequencing newly replicated DNA reveals widespread plasticity in human replication timing.

    abstract::Faithful transmission of genetic material to daughter cells involves a characteristic temporal order of DNA replication, which may play a significant role in the inheritance of epigenetic states. We developed a genome-scale approach--Repli Seq--to map temporally ordered replicating DNA using massively parallel sequenc...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0912402107

    authors: Hansen RS,Thomas S,Sandstrom R,Canfield TK,Thurman RE,Weaver M,Dorschner MO,Gartler SM,Stamatoyannopoulos JA

    更新日期:2010-01-05 00:00:00

  • Photosensitized destruction of human bladder carcinoma cells treated with chlorin e6-conjugated microspheres.

    abstract::A photosensitizer conjugate, chlorin e6 (Ce6) covalently bound to 1-micron-diameter polystyrene microspheres, has been investigated in the photodynamic destruction of MGH-U1 human bladder carcinoma cells in vitro. The microspheres were taken up avidly by the carcinoma cells; confocal laser scanning fluorescence micros...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.88.4.1580

    authors: Bachor R,Shea CR,Gillies R,Hasan T

    更新日期:1991-02-15 00:00:00

  • Structural and functional analyses reveal promiscuous and species specific use of ephrin receptors by Cedar virus.

    abstract::Cedar virus (CedV) is a bat-borne henipavirus related to Nipah virus (NiV) and Hendra virus (HeV), zoonotic agents of fatal human disease. CedV receptor-binding protein (G) shares only ∼30% sequence identity with those of NiV and HeV, although they can all use ephrin-B2 as an entry receptor. We demonstrate that CedV a...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1911773116

    authors: Laing ED,Navaratnarajah CK,Cheliout Da Silva S,Petzing SR,Xu Y,Sterling SL,Marsh GA,Wang LF,Amaya M,Nikolov DB,Cattaneo R,Broder CC,Xu K

    更新日期:2019-10-08 00:00:00

  • Withdrawal of IL-7 induces Bax translocation from cytosol to mitochondria through a rise in intracellular pH.

    abstract::IL-7 functions as a trophic factor during T lymphocyte development by a mechanism that is partly based on the induction of Bcl-2, which protects cells from apoptosis. Here we report a mechanism by which cytokine withdrawal activates the prodeath protein Bax. On loss of IL-7 in a dependent cell line, Bax protein transl...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.96.25.14476

    authors: Khaled AR,Kim K,Hofmeister R,Muegge K,Durum SK

    更新日期:1999-12-07 00:00:00

  • Ligand induction of a transcriptionally active thyroid hormone receptor coactivator complex.

    abstract::Transcriptional regulation by nuclear hormone receptors is thought to involve interactions with putative cofactors that may potentiate receptor function. Here we show that human thyroid hormone receptor alpha purified from HeLa cells grown in the presence of thyroid hormone (T3) is associated with a group of distinct ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.93.16.8329

    authors: Fondell JD,Ge H,Roeder RG

    更新日期:1996-08-06 00:00:00

  • Dynamics and biological relevance of DNA demethylation in Arabidopsis antibacterial defense.

    abstract::DNA methylation is an epigenetic mark that silences transposable elements (TEs) and repeats. Whereas the establishment and maintenance of DNA methylation are relatively well understood, little is known about their dynamics and biological relevance in plant and animal innate immunity. Here, we show that some TEs are de...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1211757110

    authors: Yu A,Lepère G,Jay F,Wang J,Bapaume L,Wang Y,Abraham AL,Penterman J,Fischer RL,Voinnet O,Navarro L

    更新日期:2013-02-05 00:00:00

  • The ability of HIV type 1 to use CCR-3 as a coreceptor is controlled by envelope V1/V2 sequences acting in conjunction with a CCR-5 tropic V3 loop.

    abstract::Although infection by primary HIV type 1 (HIV-1) isolates normally requires the functional interaction of the viral envelope protein with both CD4 and the CCR-5 coreceptor, a subset of such isolates also are able to use the distinct CCR-3 receptor. By analyzing the ability of a series of wild-type and chimeric HIV-1 e...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.95.13.7682

    authors: Ross TM,Cullen BR

    更新日期:1998-06-23 00:00:00

  • Retinoblastoma gene product as a downstream target for a ceramide-dependent pathway of growth arrest.

    abstract::Ceramide, a lipid mediator, has been most closely associated with antiproliferative activities. In this study, we examine the mechanism by which ceramide induces growth suppression and the role of the retinoblastoma gene product (Rb) in this process. Withdrawal of serum from the serum-dependent MOLT-4 cells resulted i...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.92.5.1347

    authors: Dbaibo GS,Pushkareva MY,Jayadev S,Schwarz JK,Horowitz JM,Obeid LM,Hannun YA

    更新日期:1995-02-28 00:00:00

  • Oleic acid content is responsible for the reduction in blood pressure induced by olive oil.

    abstract::Numerous studies have shown that high olive oil intake reduces blood pressure (BP). These positive effects of olive oil have frequently been ascribed to its minor components, such as alpha-tocopherol, polyphenols, and other phenolic compounds that are not present in other oils. However, in this study we demonstrate th...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0807500105

    authors: Terés S,Barceló-Coblijn G,Benet M,Alvarez R,Bressani R,Halver JE,Escribá PV

    更新日期:2008-09-16 00:00:00

  • Molecular genetics of a biological clock in Drosophila.

    abstract::Ninety kilobase pairs (kb) of DNA have been isolated from the 3B region of the X chromosome of Drosophila melanogaster. Previous cytogenetic analyses have placed a gene required for rhythmic behavior (per) in this chromosomal interval. Physical characterization of a series of chromosomal rearrangements altering per lo...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.81.7.2142

    authors: Bargiello TA,Young MW

    更新日期:1984-04-01 00:00:00

  • Mice lacking N-acetylglucosaminyltransferase I activity die at mid-gestation, revealing an essential role for complex or hybrid N-linked carbohydrates.

    abstract::Eukaryotic cells require N-linked carbohydrates for survival. However, the biosynthetic intermediate Man5GlcNAc2Asn, in place of mature N-linked structures, allows glycoprotein synthesis and somatic cell growth to proceed normally. To determine whether the same would be true in a complex biological situation, the gene...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.91.2.728

    authors: Ioffe E,Stanley P

    更新日期:1994-01-18 00:00:00

  • Targeted ablation of the 25-hydroxyvitamin D 1alpha -hydroxylase enzyme: evidence for skeletal, reproductive, and immune dysfunction.

    abstract::The active form of vitamin D, 1alpha,25-dihydroxyvitamin D [1alpha,25(OH)2D], is synthesized from its precursor 25 hydroxyvitamin D [25(OH)D] via the catalytic action of the 25(OH)D-1alpha-hydroxylase [1alpha(OH)ase] enzyme. Many roles in cell growth and differentiation have been attributed to 1,25(OH)2D, including a ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.131029498

    authors: Panda DK,Miao D,Tremblay ML,Sirois J,Farookhi R,Hendy GN,Goltzman D

    更新日期:2001-06-19 00:00:00

  • Targeting cell division: small-molecule inhibitors of FtsZ GTPase perturb cytokinetic ring assembly and induce bacterial lethality.

    abstract::FtsZ, the ancestral homolog of eukaryotic tubulins, is a GTPase that assembles into a cytokinetic ring structure essential for cell division in prokaryotic cells. Similar to tubulin, purified FtsZ polymerizes into dynamic protofilaments in the presence of GTP; polymer assembly is accompanied by GTP hydrolysis. We used...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0404439101

    authors: Margalit DN,Romberg L,Mets RB,Hebert AM,Mitchison TJ,Kirschner MW,RayChaudhuri D

    更新日期:2004-08-10 00:00:00

  • The role of SOS and flap processing in microsatellite instability in Escherichia coli.

    abstract::Mutations affecting mismatch repair result in elevated frequencies of microsatellite length alteration in prokaryotes and eukaryotes. However, the finding that microsatellite instability is found often in cells with a functional mismatch repair system prompted a search for other factors of tract alteration. In the pre...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.95.17.10003

    authors: Morel P,Reverdy C,Michel B,Ehrlich SD,Cassuto E

    更新日期:1998-08-18 00:00:00

  • Empirical laws of survival and evolution: their universality and implications.

    abstract::Presented analysis of human and fly life tables proves that with the specified accuracy their entire survival and mortality curves are uniquely determined by a single point (e.g., by the birth mortality q(0)), according to the law, which is universal for species as remote as humans and flies. Mortality at any age decr...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.96.26.15368

    authors: Azbel' MY

    更新日期:1999-12-21 00:00:00

  • Transcription of a subset of human class II major histocompatibility complex genes is regulated by a nucleoprotein complex that contains c-fos or an antigenically related protein.

    abstract::Transcriptional regulation of the human major histocompatibility complex class II genes requires at least two upstream elements, the X and Y boxes, located in the -50- to -150-base-pair region of all class II promoters. The DRA and DPB promoters contain phorbol ester-responsive elements overlapping the 3' side of thei...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.88.10.4304

    authors: Ono SJ,Bazil V,Levi BZ,Ozato K,Strominger JL

    更新日期:1991-05-15 00:00:00

  • Dual role of lignin in plant litter decomposition in terrestrial ecosystems.

    abstract::Plant litter decomposition is a critical step in the formation of soil organic matter, the mineralization of organic nutrients, and the carbon balance in terrestrial ecosystems. Biotic decomposition in mesic ecosystems is generally negatively correlated with the concentration of lignin, a group of complex aromatic pol...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0909396107

    authors: Austin AT,Ballaré CL

    更新日期:2010-03-09 00:00:00

  • Cats use hollow papillae to wick saliva into fur.

    abstract::The cat tongue is covered in sharp, rear-facing spines called papillae, the precise function of which is a mystery. In this combined experimental and theoretical study, we use high-speed film, grooming force measurements, and computed tomography (CT) scanning to elucidate the mechanism by which papillae are used to gr...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1809544115

    authors: Noel AC,Hu DL

    更新日期:2018-12-04 00:00:00

  • Development of vivo of genetic variability of simian immunodeficiency virus.

    abstract::Rapid development of genetic variability may contribute to the pathogenicity of lentiviruses as it may allow escape from immune surveillance and/or from suppression of virus replication. Although apathogenic in African green monkeys, simian immunodeficiency virus isolated from African green monkeys is shown to display...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.88.18.8126

    authors: Baier M,Dittmar MT,Cichutek K,Kurth R

    更新日期:1991-09-15 00:00:00

  • Transgenic mice for interleukin 3 develop motor neuron degeneration associated with autoimmune reaction against spinal cord motor neurons.

    abstract::Interleukin 3 (IL-3) stimulates the proliferation and differentiation of various haematopoietic progenitor cells. Recently, IL-3 and other cytokines were reported to exert a neurotrophic activity and to be associated with neurological disorders, suggesting their complex role in the central nervous system. We now show ...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.95.19.11354

    authors: Chavany C,Vicario-Abejón C,Miller G,Jendoubi M

    更新日期:1998-09-15 00:00:00

  • Vaccination can drive an increase in frequencies of antibiotic resistance among nonvaccine serotypes of Streptococcus pneumoniae.

    abstract::The bacterial pathogen Streptococcus pneumoniae is a major public health concern, being responsible for more than 1.5 million deaths annually through pneumonia, meningitis, and septicemia. Available vaccines target only a subset of serotypes, so vaccination is often accompanied by a rise in the frequency of nonvaccine...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.1718712115

    authors: Obolski U,Lourenço J,Thompson C,Thompson R,Gori A,Gupta S

    更新日期:2018-03-20 00:00:00

  • Ab-induced ectodomain shedding mediates hepatocyte growth factor receptor down-regulation and hampers biological activity.

    abstract::Targeting tyrosine kinase receptors (RTKs) with specific Abs is a promising therapeutic approach for cancer treatment, although the molecular mechanism(s) responsible for the Abs' biological activity are not completely known. We targeted the transmembrane RTK for hepatocyte growth factor (HGF) with a monoclonal Ab (DN...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.0508156103

    authors: Petrelli A,Circosta P,Granziero L,Mazzone M,Pisacane A,Fenoglio S,Comoglio PM,Giordano S

    更新日期:2006-03-28 00:00:00

  • Conservation of sequence and structure flanking the mouse and human beta-globin loci: the beta-globin genes are embedded within an array of odorant receptor genes.

    abstract::In mouse and human, the beta-globin genes reside in a linear array that is associated with a positive regulatory element located 5' to the genes known as the locus control region (LCR). The sequences of the mouse and human beta-globin LCRs are homologous, indicating conservation of an essential function in beta-globin...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.96.9.5129

    authors: Bulger M,van Doorninck JH,Saitoh N,Telling A,Farrell C,Bender MA,Felsenfeld G,Axel R,Groudine M

    更新日期:1999-04-27 00:00:00

  • Obesity and hyperleptinemia in metallothionein (-I and -II) null mice.

    abstract::Metallothionein (MT) has several putative roles in metal detoxification, in Zn and Cu homeostasis, in scavenging free radicals, and in the acute phase response. Mice of mixed 129/Ola and C57BL/6J background with targeted disruption of MT-I and MT-II genes are more sensitive to toxic metals and oxidative stress. We not...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.95.1.358

    authors: Beattie JH,Wood AM,Newman AM,Bremner I,Choo KH,Michalska AE,Duncan JS,Trayhurn P

    更新日期:1998-01-06 00:00:00

  • Configurations for a proof of principle stellarator experiment.

    abstract::One of the serious limitations of tokamaks as reactors is the occurrence of disruptions. Stellarators designed by advanced computational methods provide an attractive alternative for a major experiment in magnetic fusion research. Configurations with approximate two-dimensional magnetic symmetry have been found with h...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.97.3.972

    authors: Garabedian PR

    更新日期:2000-02-01 00:00:00

  • Induction of DNA synthesis in terminally differentiated myotubes by the activation of the src gene of Rous sarcoma virus.

    abstract::Mononucleated myogenic cells from 11-day-old chicken embryos were infected with tsLA24 or tsNY68, temperature-sensitive transformation mutants of Rous sarcoma virus. The infected mononucleated myogenic cells were incubated at the nonpermissive temperature (41 degrees C) and allowed to develop into multinucleated myotu...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.75.11.5501

    authors: Kobayashi N,Kaji A

    更新日期:1978-11-01 00:00:00

  • Genotypic variation within asexual lineages of Taraxacum officinale.

    abstract::Restriction site variation in DNA that encodes rRNA (rDNA) was surveyed among 714 offspring within 31 lineages (26 genotypes) of obligate asexually reproducing Taraxacum officinale (dandelions). Although clonal offspring are expected, plants with nonparental rDNA were produced from two parents that were themselves sib...

    journal_title:Proceedings of the National Academy of Sciences of the United States of America

    pub_type: 杂志文章

    doi:10.1073/pnas.87.3.998

    authors: King LM,Schaal BA

    更新日期:1990-02-01 00:00:00