Differential astrocyte and oligodendrocyte vulnerability in murine Creutzfeldt-Jakob disease.

Glial vulnerability to prions is assessed in murine Creutzfeldt-Jakob disease (CJD) using the tg340 mouse line expressing four-fold human PrP M129 levels on a mouse PrP null background at different days following intracerebral inoculation of sCJD MM1 brain tissues homogenates. The mRNA expression of several astrocyte markers, including glial fibrillary acidic protein (), aquaporin-4 (), solute carrier family 16, member 4 (), mitochondrial pyruvate carrier 1 () and solute carrier family 1, member 2 (glial high-affinity glutamate transporter, ) increases at 120 and 180 dpi. In contrast, the mRNA expression of oligodendrocyte and myelin markers oligodendrocyte transcription factor 1 (, neural/glial antigen 2 (), solute carrier family 16, member 1 (), myelin basic protein (), myelin oligodendrocyte glycoprotein () and proteolipid protein 1 () is preserved.

Conjunctival calcinosis circumscripta in a dog: Treatment combining surgical resection and amniotic membrane grafting.

This report describes the clinical features, histopathology, and surgical treatment of a case of conjunctival calcification in a 5-month-old female English Setter, referred with a history of recurrent conjunctivitis in the right eye (OD). The ophthalmic findings were limited to multifocal white plaques embedded in a markedly inflamed conjunctiva of the eyelids and the anterior nictitating membrane OD. Calcification was suspected.

Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice.

Classical bovine spongiform encephalopathy (BSE) is the only zoonotic prion disease described to date. Although the zoonotic potential of atypical BSE prions have been partially studied, an extensive analysis is still needed. We conducted a systematic study by inoculating atypical BSE isolates from different countries in Europe into transgenic mice overexpressing human prion protein (PrP): TgMet, TgMet/Val, and TgVal.

Infectivity in bone marrow from sporadic CJD patients.

Prion infectivity was recently identified in the blood of both sporadic and variant Creutzfeldt-Jakob disease (CJD) patients. In variant CJD (vCJD), the widespread distribution of prions in peripheral tissues of both asymptomatic and symptomatic patients is likely to explain the occurrence of the observed prionaemia. However, in sporadic CJD (sCJD), prion infectivity is described to be located principally in the central nervous system.

Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients.

In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD.

Leukoreduction and blood-borne vCJD transmission risk.

Purpose Of Review: Risk assessments for transmission of variant Creutzfeldt-Jakob disease predicted that leukocyte reduction would be inefficient at preventing transmission of the disease by blood transfusion. Nevertheless, approximately 14 years ago, a significant proportion of European countries decided to implement leukocyte reduction treatment within their human blood supplies in order to mitigate the risk of human prion disease transmission.

Recent Findings: Current epidemiological studies seem to indicate that leukocyte reduction has had a positive impact on reducing the risk.

Detection of infectivity in blood of persons with variant and sporadic Creutzfeldt-Jakob disease.

We report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant Creutzfeldt-Jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic Creutzfeldt-Jakob disease. The measured infectivity levels were comparable to those reported in various animals with transmissible spongiform encephalopathies.