Category: publicaciones

Rapid generation of prion disease models using AAV-delivered PrP variants in knockout mice

Rapid generation of prion disease models using AAV-delivered PrP variants in knockout mice

Overexpression of bank vole PrP(I109) in mice induces a spontaneous atypical prion disease with sex-dependent onset, early NfL elevation, and universal prion strain permissiveness

Overexpression of bank vole PrP(I109) in mice induces a spontaneous atypical prion disease with sex-dependent onset, early NfL elevation, and universal prion strain permissiveness

Advancing prion diagnostics: full-length human E200K RT-QuIC substrate facilitates prion detection in tear fluid and improves sensitivity in cerebrospinal fluid

Advancing prion diagnostics: full-length human E200K RT-QuIC substrate facilitates prion detection in tear fluid and improves sensitivity in cerebrospinal fluid

Variably Protease-Sensitive Prionopathy: Two New Cases With Motor Neuron-Dementia Syndrome

Variably Protease-Sensitive Prionopathy: Two New Cases With Motor Neuron-Dementia Syndrome Investigadores de CIC bioGUNE y ATLAS Molecular Pharma, en colaboración con centros europeos, han descrito dos nuevos casos de Variably Protease-Sensitive Prionopathy (VPSPr), una forma excepcional de enfermedad priónica que se manifestó como un síndrome de motoneurona superior y demencia frontotemporal. Los pacientes desarrollaron alteraciones […]

Spontaneous generation of diverse recombinant prion strains: sulfated glycan cofactors facilitate strain emergence but do not determine specific strain properties

Spontaneous generation of diverse recombinant prion strains: sulfated glycan cofactors facilitate strain emergence but do not determine specific strain properties ¿Qué pasaría si pudiéramos observar el instante en que un prion “nace” de forma espontánea? Este estudio se acerca como nunca antes a ese misterio. Utilizando distintos glicanos sulfatados —heparina, condroitín sulfato y pentosano polisulfato— […]

The Risk of Transmission of Genetic Prion Diseases is Greater Than 50%

The Risk of Transmission of Genetic Prion Diseases is Greater Than 50% Durante décadas se ha asumido que las enfermedades priónicas hereditarias siguen las reglas básicas de Mendel: un 50% de probabilidad de transmitir la mutación a cada hijo. Pero ¿y si ese pilar de la genética clásica estuviera equivocado? Este estudio revela un hallazgo […]

A comprehensive phylogeny of mammalian PRNP gene reveals no influence of prion misfolding propensity on the evolution of this gene

The study shows that prion properties are conserved without post-translational modifications. An international team led by researchers from IRTA-CReSA and CIC bioGUNE has shown that the fastest known prion strain, CWD-vole, maintains its properties even in the absence of glycosylation and membrane anchoring. Using...

Effect of primary structural variation on cervid prion protein in flexibility, stability, and spontaneous misfolding propensity

Cofactors favour prion misfolding, but are not essential to generate infectivity A multidisciplinary team led by researchers from CIC bioGUNE and in collaboration with partners from the NEURO-COOP project, has demonstrated that the prion protein [...]

Characterization of prion strains and peripheral prion infectivity patterns in E200K genetic CJD patients

Researchers from the Centre for Encephalopathies and Emerging Communicable Diseases of the University of Zaragoza, within the framework of the NEURO-COOP project, have managed to detect prions (PrP^Sc) in the oocytes and ovaries of sheep naturally infected with classical scrapie, using the ultra-sensitive PMCA (Protein Misfolding Cyclic Amplification) technique.

Detection of prions in oocytes and ovaries of ewes naturally infected with classical scrapie

Researchers from the Centre for Encephalopathies and Emerging Communicable Diseases of the University of Zaragoza, within the framework of the NEURO-COOP project, have managed to detect prions (PrP^Sc) in the oocytes and ovaries of sheep naturally infected with classical scrapie, using the ultra-sensitive PMCA (Protein Misfolding Cyclic Amplification) technique.