Review: Laboratory diagnosis and surveillance of Creutzfeldt-Jakob disease

Lee, Jeongmin; Hyeon, Jae Wook; Kim, Su Yeon; Hwang, Kyu-Jam; Ju, Young Ran; Ryou, Chongsuk

doi:10.1002/jmv.24004

Cited by 25 publications

(25 citation statements)

References 81 publications

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“…Diagnosis of human prion disease is based on multiple criteria (reviewed in [39]). Clinical and neurological surveys are key to diagnosing prion disease.…”

Section: Approaches For Diagnosis Of Prion Diseasementioning

confidence: 99%

Prion Diagnosis: Application of Real-Time Quaking-Induced Conversion

Kang

Rahim

et al. 2017

BioMed Research International

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Prions composed of pathogenic scrapie prion protein (PrPSc) are infectious pathogens that cause progressive neurological conditions known as prion diseases or transmissible spongiform encephalopathies. Although these diseases pose considerable risk to public health, procedures for early diagnosis have not been established. One of the most recent attempts at sensitive and specific detection of prions is the real-time quaking-induced conversion (RT-QuIC) method, which measures the activity of PrPSc aggregates or amyloid formation triggered by PrPSc seeds in the presence of recombinant PrP. In this review, we summarize prions, prion diseases, and current approaches to diagnosis, including the principle, conditions for assay performance, and current diagnostic applications of RT-QuIC.

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“…Diagnosis of human prion disease is based on multiple criteria (reviewed in [39]). Clinical and neurological surveys are key to diagnosing prion disease.…”

Section: Approaches For Diagnosis Of Prion Diseasementioning

confidence: 99%

Prion Diagnosis: Application of Real-Time Quaking-Induced Conversion

Kang

Rahim

et al. 2017

BioMed Research International

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“…Therefore, in combination with the transmissible nature of the disease, autopsy or brain biopsy cannot be performed due to limited medical resources in China and in India (18). Given this diagnostic dilemma, nonpathological methods are sought as a reference standard, and active surveillance and effective diagnosis of CJD are critical (19).…”

Section: Discussionmentioning

confidence: 99%

Three sporadic cases of Creutzfeldt-Jakob disease in China and their clinical analysis

Wang

Liu

et al. 2017

Experimental and Therapeutic Medicine

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Abstract. The present study described the characteristics of three cases of Creutzfeldt-Jakob disease (CJD) in China and analyzed their clinical presentations. The clinical information of the three cases was collected and analyzed. Blood and cerebrospinal fluid (CSF) specimens of the patients were collected for detection of the prion protein (PRNP) gene and 14-3-3 protein levels. Dynamic changes of electroencephalograms (EEGs) and brain magnetic resonance images (MRIs) were also observed. All the three cases were sporadic CJD cases. They presented with symptoms including hyposthenia, progressive memory loss, truncal and limb ataxia, dysarthria, lowered vision acuity, bucking, language disorders, myoclonia and akinetic mutism state. One of the three cases was associated with a prolonged duration of >6 years. The EEG of two cases showed slow biphasic waves. The diffusion-weighted MRI sequence revealed abnormal hyperintensity and bilateral ribboning in the cortex. Two patients tested positive for the 14-3-3 protein in the CSF. All patients were of methionine homozygosity at codon 129 in the gene encoding PRNP protein and one patient had a mutation. The CJD cases showed differences in terms of symptoms and disease duration. Subacute onset was common and with attentive nursing and supportive treatments, one of the patients had a prolonged survival time of >6 years. IntroductionCreutzfeldt-Jakob disease (CJD) is a progressive neurodegenerative disorder that is caused by prion protein deposition in the central nervous system. As the most common type of human transmissible spongiform encephalopathy, CJD is incurable and fatal with a duration from a few months to two years (1). Typical CJD triad symptoms include progressive dementia, myoclonus and electroencephalographic (EEG) changes (periodic triphasic sharp wave complexes) (2). Traditionally, CJD is classified as sporadic (sCJD), familial (fCJD), iatrogenic (iCJD) and variant CJD (vCJD) (3). Different subtypes are associated with specific clinical symptoms and pathological features (4,5). sCJD, accounting for ~85% of all cases worldwide, is categorized into six subtypes according to the methionine (M)/valine (V) polymorphism at codon 129 of the PRNP gene (6,7). fCJD accounts for 5-10% of all cases and is an autosomal dominant disorder associated with various mutations of the PRNP gene (8,9). The main causes of iCJD include contaminated human hormone supplements, corneal transplantation and transplantation of cadaveric dura mater (10). At present, vCJD, which is transmitted from cows with bovine spongiform encephalopathy to humans, mainly affects younger adults in Europe with different clinical, etiological and neuropathological features as well as risk factors compared with other types of CJD (11-13).Although final diagnosis of CJD requires neuropathological biopsy examination of affected brain tissues, nonpathological methods are currently used worldwide for the early diagnosis and prevention of CJD (14). At present, the diagnosis of CJD is frequently delayed or CJD ...

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“…PK-resistant PrP Sc included in the pellet was detected by western blotting using anti-PrP antibody clone 5C6. Separately, an aliquot (~ 30 μg) of ScN2a or N2a cell lysate was analyzed without PK digestion by western blotting for total PrP using anti [14] PrP antibody clones SAF32, 6D11 and 5C6. β-actin and βIII tubulin were also detected as the loading control.…”

Section: Methodsmentioning

confidence: 99%

“…These diseases manifest progressive neuronal degeneration and subsequent death [11]. Currently, no therapy is available for prion diseases including Creutzfeldt-Jakob disease in humans [13, 14]. The prion agents are composed of the disease-causing, scrapie prion protein (PrP Sc ) only and propagate through macromolecular interaction between the normal, cellular prion protein (PrP C ) and PrP Sc followed by conformational conversion of PrP C to PrP Sc [11].…”

Section: Introductionmentioning

confidence: 99%

Effect of poly-L-arginine in inhibiting scrapie prion protein of cultured cells

et al. 2017

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Biological effect of poly-L-arginine (PLR), the linear homopolymer comprised of L-arginine, was investigated to determine the activity of suppressing prions. PLR decreased the level of scrapie prion protein (PrPSc) in cultured cells permanently infected with prions in a concentration dependent manner. The PrPSc inhibition efficacy of PLR was greater than that of another prion suppressant poly-L-lysine (PLK) in a molecular mass-dependent fashion. The effective concentration of PLR to inhibit prions was achieved safely below the cytotoxic concentrations and overall cytotoxicity of PLR was similar to that of PLK. PLR did not alter the cellular prion protein (PrPC) level and was unable to change the states of preformed recombinant PrP aggregates and PrPSc from prion-infected cells. These data eliminate the possibility that the action mechanism of PLR is through removal of PrPC and pre-existing PrPSc. However, PLR formed complexes with plasminogen that stimulates prion propagation via conversion of PrPC to the misfolded isoform, PrPSc. The plasminogen-PLR complex demonstrated the greater positive surface charge values than the similar complex with PLK, raising the possibility that PLR interferes with the role of cofactor for PrPSc generation better than PLK.

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Review: Laboratory diagnosis and surveillance of Creutzfeldt-Jakob disease

Cited by 25 publications

References 81 publications

Prion Diagnosis: Application of Real-Time Quaking-Induced Conversion

Prion Diagnosis: Application of Real-Time Quaking-Induced Conversion

Three sporadic cases of Creutzfeldt-Jakob disease in China and their clinical analysis

Effect of poly-L-arginine in inhibiting scrapie prion protein of cultured cells

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