Delayed progression of prion disease in mice by polyarginine-facilitated prevention of PrP propagation in the spleen.

Prions are infective agents composed of abnormally folded prion proteins (PrP), which are pathogenic isoforms of normal cellular prion proteins (PrP) that cause incurable, transmissible, neurodegenerative conditions in mammals called prion diseases. The spread of PrP within a host is facilitated by the lymphoreticular system, which uptakes and propagates PrP in the periphery and transmits them to the central nervous system. Our previous study showed that poly-l-arginine (PLR), a cationic amino acid polymer, inhibits PrP accumulation in neuroblastoma cells with persistent prion infection (ScN2a).

Detection of chronic wasting disease prions in the farm soil of the Republic of Korea.

Chronic wasting disease (CWD) is a highly contagious prion disease occurring in free-ranging and farmed cervids. CWD continues to spread uncontrolled across North America, and cases continue to be detected almost every year in the Republic of Korea. CWD-infected animals contaminate the soil by releasing infectious prions through their excreta, and shed prions accumulate and remain infectious in the soil for years.

Identification of a novel risk factor for chronic wasting disease (CWD) in elk: S100G single nucleotide polymorphism (SNP) of the prion protein gene (PRNP).

Prion diseases are fatal and malignant infectious encephalopathies induced by the pathogenic form of prion protein (PrP) originating from benign prion protein (PrP). A previous study reported that the M132L single nucleotide polymorphism (SNP) of the prion protein gene (PRNP) is associated with susceptibility to chronic wasting disease (CWD) in elk. However, a recent meta-analysis integrated previous studies that did not find an association between the M132L SNP and susceptibility to CWD.

Autoclave treatment fails to completely inactivate DLB alpha-synuclein seeding activity.

Synucleinopathies are characterized by the deposition of alpha-synuclein (α-syn) aggregates in brain tissue. Pathological α-syn aggregates propagate in a prion-like manner and display prion-like biochemical properties. Using RT-QuIC, we measured α-syn seeding activity from brains of Dementia with Lewy body (DLB) patients post autoclave.

Association Study of the M132L Single Nucleotide Polymorphism With Susceptibility to Chronic Wasting Disease in Korean Elk: A Meta-Analysis.

Chronic wasting disease (CWD) is a deleterious brain proteinopathy caused by a pathogenic form of prion protein (PrP), which is converted from a benign form of prion protein (PrP) encoded by the prion protein gene (). In elk, the M132L single nucleotide polymorphism (SNP) of the gene likely plays a pivotal role in susceptibility to CWD. However, the association of the M132L SNP with susceptibility to CWD has not been evaluated in Korean elk to date.

In-depth examination of PrP in Holstein cattle carrying the E211K somatic mutation of the bovine prion protein gene (PRNP).

Prion diseases are transmissible spongiform encephalopathies caused by deleterious prion protein (PrP ) derived from normal prion protein (PrP ), which is encoded by the prion protein gene (PRNP). We performed an in-depth examination to detect PrP by using enzyme immunoassay (EIA), real-time quaking-induced conversion reactions (RT-QuIC) and protein misfolding cyclic amplification (PMCA) in nine brain tissues derived from three Holstein cattle carrying the E211K somatic mutation of the bovine PRNP gene. The EIA, RT-QuIC and PMCA analyses were not able to detect the PrP band in any tested samples.

The complete mitochondrial genome of (Erxleben, 1777), as a model species of Chronic Wasting Disease (CWD).

(Erxleben, 1777) has been used as a model species of Chronic Wasting Disease (CWD). We completed the mitochondrial genome of , susceptible to the CWD. Its length is 16,428 bp, identical to the previous mitochondrial genome of , and 37 genes (13 protein-coding genes, two rRNAs, and 22 tRNAs) were identified.

The first complete mitogenome of (Merriam, 1905).

(Merriam, 1905) is one of the subspecies of elk distributed only in California, USA. We completed the first mitogenome of . Its length is 16,428 bp, which is in middle among 24 available mitogenomes.

Experimental oral transmission of chronic wasting disease to sika deer ().

Chronic wasting disease (CWD) affects a broad array of cervid species and continues to be detected in an expanding geographic range. Initially introduced into the Republic of Korea through the importation of CWD-infected elk (), additional cases of CWD were subsequently detected in farmed Korean elk and sika deer (). Wild and farmed sika deer are found in many regions of Asia, North America, and Europe, although natural transmission to this species has not been detected outside of the Republic of Korea.

Identification of the prion-related protein gene (PRNT) sequences in various species of the Cervidae family.

Chronic wasting disease (CWD) is caused by abnormal deleterious prion protein (PrP), and transmissible spongiform encephalopathy occurs in the Cervidae family. In recent studies, the susceptibility of prion disease has been affected by polymorphisms of the prion gene family. However, the study of the prion-related protein gene (PRNT) is rare, and the DNA sequence of this gene was not fully reported in all Cervidae families.

Outbreak of African swine fever in South Korea, 2019.

African swine fever, a fatal haemorrhagic disease of swine, was confirmed in domestic pigs for the first time in South Korea in September 2019. The causative virus belonged to the p72 genotype II and had an additional tandem repeat sequence in the intergenic region (IGR) between the I73R and I329L.

Sodium hydroxide treatment effectively inhibits PrP replication in farm soil.

Chronic wasting disease (CWD) agents are shed into biological samples, facilitating their horizontal transmission between cervid species. Once prions enter the environment, binding of PrP by soil particles may maintain them near the soil surface, posing a challenge for decontamination. A 2 N sodium hydroxide (NaOH) or 2% sodium hypochlorite (NaClO) solution is traditionally recommended for prion decontamination of equipment and surfaces.

Application of the fragment molecular orbital method to discover novel natural products for prion disease.

Conformational conversion of the normal cellular isoform of the prion protein PrP into an infectious isoform PrP causes pathogenesis in prion diseases. To date, numerous antiprion compounds have been developed to block this conversion and to detect the molecular mechanisms of prion inhibition using several computational studies. Thus far, no suitable drug has been identified for clinical use.

Establishment of a Madin-Darby bovine kidney cell line expressing anchorless bovine prion protein.

Enzyme-linked immunosorbent assay (ELISA) performed using extensively purified bacterially expressed bovine prion protein (PrP) shows decreased cross-reactivity. We generated a transduced Madin-Darby bovine kidney (MDBK) cell line continuously expressing glycosylphosphatidylinositol (GPI)-anchorless bovine PrP (designated as MDBK ∆GPI protein) by using a lentiviral expression system. The present study also described the method for purifying bovine PrP through sequential culturing without the need for complex purification protocol.

Biological and biochemical characterization of M2B cells: Classical BSE prion is conserved in transgenic mice overexpressing bovine prion protein gene.

M2B cells with persistent classical bovine spongiform encephalopathy (C-BSE) have been established previously. In this study, we performed strain characterization of the M2B cell line in bovine PrP overexpressing mice (Tg 1896). Mice intracranially inoculated with M2B cells and C-BSE survived for 451 ± 7 and 465 ± 31 d post inoculation, respectively.

Effect of bihemispheric anodal transcranial direct current stimulation for dysphagia in chronic stroke patients: A randomized clinical trial.

Objective: To investigate whether bihemispheric anodal transcranial direct current stimulation (tDCS) with conventional dysphagia therapy could improve swallowing function in chronic stroke patients with dysphagia.

Design: Randomized controlled trial.

Subjects: Twenty-six patients with dysphagia for at least 6 months post-stroke were randomly assigned into: (i) bihemispheric anodal tDCS group; or (ii) sham group.

Experimental chronic wasting disease in wild type VM mice.

Chronic wasting disease (CWD) is a naturally occurring prion disease in North American deer (Odocoileus species), Rocky mountain elk (Cervus elaphus nelsoni) and moose (Alces alces). The disease was first confirmed in the Republic of Korea in 2001, and subsequent cases were diagnosed in 2004, 2005 and 2010. The experimental host range of CWD includes ferrets, several species of voles, white-footed mice, deer mice and Syrian golden hamsters.

Control of foot-and-mouth disease during 2010-2011 epidemic, South Korea.

An outbreak of foot-and-mouth disease caused by serotype O virus occurred in cattle and pigs in South Korea during November 2010-April 2011. The highest rates of case and virus detection were observed 44 days after the first case was detected. Detection rates declined rapidly after culling and completion of a national vaccination program.

Strain characterization of the Korean CWD cases in 2001 and 2004.

Chronic wasting disease (CWD) has been recognized as a naturally occurring prion disease in North American deer (Odocoileus species), Rocky Mountain elk (Cervus elaphus nelsoni) and moose (Alces alces). The disease was confirmed only in elk in the Republic of Korea in 2001, 2004 and 2005. Epidemiological investigations showed that CWD was introduced via importation of infected elk from Canada between 1994 and 1997.

Establishment of a cell line persistently infected with chronic wasting disease prions.

Elk prion protein (PrP(C)) has been confirmed to be capable of rendering rabbit epithelial RK13 cells permissive to temporal infection by chronic wasting disease (CWD) prions. The present study satisfactorily generated persistently CWD prion-affected RK13 cells (RKC1-11) using elk PrP(C) expressing cells (elkRK13) that were generated via the lentiviral expression system with high efficiency. The elkRK13 cells have been shown to be permissive to accumulation of abnormal isoforms of prion protein (PrP(Sc)) resulting from CWD prions up to 97 serial passages thus far.

Isolated bilateral abducens nerves palsy due to traumatic delicate arteriovenous fistula.

Background: Ocular motor dysfunction is common in patients with head trauma. Also, traumatic lateral gaze palsy is usually associated with brain stem lesion, peripheral nerve injury with or without basilar skull fracture and lateral rectus muscle injury or entrapment. However, isolated bilateral abducens nerve palsy is extremely rare.