Jorge M. Charco
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View article: A comprehensive phylogeny of mammalian PRNP gene reveals no influence of prion misfolding propensity on the evolution of this gene
A comprehensive phylogeny of mammalian PRNP gene reveals no influence of prion misfolding propensity on the evolution of this gene Open
Prion diseases are invariably fatal neurodegenerative diseases that affect some mammalian species, including humans. These diseases are caused by the misfolding of the cellular prion protein (PrP C ) into a pathologic isoform (PrP Sc ). Th…
View article: Cofactors facilitate bona fide prion misfolding in vitro but are not necessary for the infectivity of recombinant murine prions
Cofactors facilitate bona fide prion misfolding in vitro but are not necessary for the infectivity of recombinant murine prions Open
Prion diseases, particularly sporadic cases, pose a challenge due to their complex nature and heterogeneity. The underlying mechanism of the spontaneous conversion from PrP C to PrP Sc , the hallmark of prion diseases, remains elusive. To …
View article: Analysis of a large case series of fatal familial insomnia to determine tests with the highest diagnostic value
Analysis of a large case series of fatal familial insomnia to determine tests with the highest diagnostic value Open
Fatal familial insomnia (FFI) is a rare prionopathy with unusually high incidence in the Basque Country. We report detailed data on clinical, diagnostic, histopathological, and biochemical characteristics of a recent FFI case series. The B…
View article: Glycans are not necessary to maintain the pathobiological features of bovine spongiform encephalopathy
Glycans are not necessary to maintain the pathobiological features of bovine spongiform encephalopathy Open
The role of the glycosylation status of PrP C in the conversion to its pathological counterpart and on cross-species transmission of prion strains has been widely discussed. Here, we assessed the effect on strain characteristics of bovine …
View article: Description of the first Spanish case of Gerstmann–Sträussler–Scheinker disease with A117V variant: clinical, histopathological and biochemical characterization
Description of the first Spanish case of Gerstmann–Sträussler–Scheinker disease with A117V variant: clinical, histopathological and biochemical characterization Open
Gerstmann–Sträussler–Scheinker disease (GSS) is a rare neurodegenerative illness that belongs to the group of hereditary or familial Transmissible Spongiform Encephalopathies (TSE). Due to the presence of different pathogenic alterations i…
View article: The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data
The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data Open
Background Fifty-three percent of all cases of malaria in the Americas in 2019 came from Venezuela, where the epidemic is heavily focused south of the Orinoco river, and where most of the country’s Amerindian groups live. Although the dise…
View article: Improving the Pharmacological Properties of Ciclopirox for Its Use in Congenital Erythropoietic Porphyria
Improving the Pharmacological Properties of Ciclopirox for Its Use in Congenital Erythropoietic Porphyria Open
Congenital erythropoietic porphyria (CEP), also known as Günther’s disease, results from a deficient activity in the fourth enzyme, uroporphyrinogen III synthase (UROIIIS), of the heme pathway. Ciclopirox (CPX) is an off-label drug, topica…
View article: Homozygous R136S mutation in PRNP gene causes recessive inherited early onset prion disease
Homozygous R136S mutation in PRNP gene causes recessive inherited early onset prion disease Open
Background: More than 40 pathogenic heterozygous PRNP mutations causing inherited prion diseases have been identified to date. Recessive inherited prion disease has not been described to date. Methods: We describe the clinical and neuropat…
View article: Detection of chronic wasting disease in mule and white-tailed deer by RT-QuIC analysis of outer ear
Detection of chronic wasting disease in mule and white-tailed deer by RT-QuIC analysis of outer ear Open
Efforts to contain the spread of chronic wasting disease (CWD), a fatal, contagious prion disease of cervids, would be aided by the availability of additional diagnostic tools. RT-QuIC assays allow ultrasensitive detection of prion seeds i…
View article: Additional file 2 of The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data
Additional file 2 of The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data Open
Additional file 2. Malaria incidence in indigenous patients and relative risk compared to non-indigenous population.
View article: Additional file 3 of The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data
Additional file 3 of The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data Open
Additional file 3. Estimated indigenous population and growth rates.
View article: Additional file 4 of The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data
Additional file 4 of The malaria burden of Amerindian groups of three Venezuelan states: a descriptive study based on programmatic data Open
Additional file 4. Getis-Ord’s Gi* Analysis for clustering of malaria cases (2014–2018).
View article: A Novel, Reliable and Highly Versatile Method to Evaluate Different Prion Decontamination Procedures
A Novel, Reliable and Highly Versatile Method to Evaluate Different Prion Decontamination Procedures Open
Transmissible spongiform encephalopathies (TSEs) are a group of invariably fatal neurodegenerative disorders. The causal agent is an aberrantly folded isoform (PrPSc or prion) of the endogenous prion protein (PrPC) which is neurotoxic and …
View article: Detection of Pathognomonic Biomarker PrPSc and the Contribution of Cell Free-Amplification Techniques to the Diagnosis of Prion Diseases
Detection of Pathognomonic Biomarker PrPSc and the Contribution of Cell Free-Amplification Techniques to the Diagnosis of Prion Diseases Open
Transmissible spongiform encephalopathies or prion diseases are rapidly progressive neurodegenerative diseases, the clinical manifestation of which can resemble other promptly evolving neurological maladies. Therefore, the unequivocal ante…
View article: Dogs are resistant to prion infection, due to the presence of aspartic or glutamic acid at position 163 of their prion protein
Dogs are resistant to prion infection, due to the presence of aspartic or glutamic acid at position 163 of their prion protein Open
Unlike other species, prion disease has never been described in dogs even though they were similarly exposed to the bovine spongiform encephalopathy (BSE) agent. This resistance prompted a thorough analysis of the canine PRNP gene and the …
View article: Optimization of techniques and procedures for the production and management of live feed used in the larval culture of the freshwater shrimp Cryphiops caementarius (Decapoda: Palaemonidae)
Optimization of techniques and procedures for the production and management of live feed used in the larval culture of the freshwater shrimp Cryphiops caementarius (Decapoda: Palaemonidae) Open
The variation in saline requirements during larval development of Cryphiops caementarius establishes a particular coexistence in the culture systems between the shrimp larvae, the microalgae and Artemia nauplii. Although the technology for…
View article: Development of a new largely scalable in vitro prion propagation method for the production of infectious recombinant prions for high resolution structural studies
Development of a new largely scalable in vitro prion propagation method for the production of infectious recombinant prions for high resolution structural studies Open
The resolution of the three-dimensional structure of infectious prions at the atomic level is pivotal to understand the pathobiology of Transmissible Spongiform Encephalopathies (TSE), but has been long hindered due to certain particularit…
View article: The amino acid residue in position 163 of canine PrP<sup>C</sup> is critical to the exceptional resistance of dogs to prion infections: evidence from transgenic mouse models
The amino acid residue in position 163 of canine PrP<sup>C</sup> is critical to the exceptional resistance of dogs to prion infections: evidence from transgenic mouse models Open
Unlike other species, such as cattle, cats or humans, prion disease has never been described in dogs, even though they were similarly exposed to the bovine spongiform encephalopathy (BSE) agent. This resistance prompted a thorough analysis…
View article: Molecular Detection and Species Determination of Malaria Parasites, Venezuela
Molecular Detection and Species Determination of Malaria Parasites, Venezuela Open
In southeastern Venezuela, malaria cases have increased since 2013. We found that 46% of 352 blood samples from symptomatic patients in 1 municipality tested positive for Plasmodium spp. In addition, the number of cases increased by 10 tim…
View article: Repurposing ciclopirox as a pharmacological chaperone in a model of congenital erythropoietic porphyria
Repurposing ciclopirox as a pharmacological chaperone in a model of congenital erythropoietic porphyria Open
Congenital erythropoietic porphyria is a rare autosomal recessive disease produced by deficient activity of uroporphyrinogen III synthase, the fourth enzyme in the heme biosynthetic pathway. The disease affects many organs, can be life-thr…
View article: Repurposing ciclopirox as a pharmacological chaperone in a model of congenital erythropoietic porphyria
Repurposing ciclopirox as a pharmacological chaperone in a model of congenital erythropoietic porphyria Open
The off-patent marketed antifungal ciclopirox improves symptoms in a mouse model of congenital erythropoietic porphyria.
View article: Recombinant PrP and Its Contribution to Research on Transmissible Spongiform Encephalopathies
Recombinant PrP and Its Contribution to Research on Transmissible Spongiform Encephalopathies Open
The misfolding of the cellular prion protein (PrPC) into the disease-associated isoform (PrPSc) and its accumulation as amyloid fibrils in the central nervous system is one of the central events in transmissible spongiform encephalopathies…