A5056268295- Prion Diseases and Protein Misfolding
- Neurological diseases and metabolism
- Trace Elements in Health
- Alcoholism and Thiamine Deficiency
- Folate and B Vitamins Research
- Escherichia coli research studies
- Viral gastroenteritis research and epidemiology
- Salmonella and Campylobacter epidemiology
- Digestive system and related health
- Enzyme Structure and Function
- Microstructure and Mechanical Properties of Steels
- RNA regulation and disease
- Bacterial Genetics and Biotechnology
- Mast cells and histamine
- Genetic factors in colorectal cancer
- Cardiac Arrhythmias and Treatments
- Animal Genetics and Reproduction
- Renal Diseases and Glomerulopathies
University of Edinburgh
2008-2023
Western General Hospital
2009-2014
Southern General Hospital
2013
Scottish National Blood Transfusion Service
2009
National Blood Transfusion Service
2009
MRC Centre for Regenerative Medicine
2009
Human-to-human transmission of Creutzfeldt-Jakob disease (CJD) has occurred through medical procedures resulting in iatrogenic CJD (iCJD). One the commonest causes iCJD was use human pituitary-derived growth hormone (hGH) to treat primary or secondary deficiency. As part a comprehensive tissue-based analysis largest cohort yet collected (35 cases) UK hGH-iCJD cases, we describe clinicopathological phenotype UK. In 33/35 cases with sufficient paraffin-embedded tissue for full pathological...
ABSTRACT Type III secretion systems of enteric bacteria enable translocation effector proteins into host cells. Secreted verotoxigenic Escherichia coli O157 strains include components a apparatus, EspA, -B, and -D, as well “effectors” such the translocated intimin receptor (Tir) mitochondrion-associated protein (Map). This research has investigated regulation LEE4 translocon proteins, in particular EspA. EspA filaments could not be detected on bacterial cell surface when E. O157:H7 was...
An atypical case of prion disease is described in a 54-year-old Dutch man, homozygous for valine at codon 129 the protein gene (<i>PRNP</i>). The clinical phenotype was characterised by progressive dementia, spastic paraplegia and sensorimotor polyneuropathy. duration 20 months. Genetic analysis <i>PRNP</i> did not reveal any abnormalities. Neuropathologically, only mild spongiform change coarse granular immunohistochemical staining abnormal protein, PrP<sup>Sc</sup>, observed, with poorly...
Creutzfeldt–Jakob disease (CJD) is the prototypic human prion that occurs most commonly in sporadic and genetic forms, but it also transmissible can be acquired through medical procedures, resulting iatrogenic CJD (iCJD). The largest numbers of iCJD cases have occurred worldwide resulted from contaminated cadaveric pituitary-derived growth hormone (hGH) its use to treat primary secondary deficiency. We report a comprehensive, tissue-based molecular analysis series UK hGH-iCJD reported date,...
Sporadic prion diseases are fatal neurodegenerative disorders characterized clinically by rapidly progressive dementia and myoclonus. Variably protease‐sensitive prionopathy (VPSPr) is a recently identified sporadic human disorder that may present with lengthy atypical clinical history. Here, we describe case of VPSPr in patient long history suspected frontotemporal (FTD). A 61‐year‐old man presented speech difficulties, including naming objects constructing multipart sentences, while there...
Summary Escherichia coli O157:H7 is a zoonotic pathogen that can express type III secretion system (TTSS) considered important for colonization and persistence in ruminants. E. strains have been shown to vary markedly levels of protein secreted using the TTSS this study has confirmed high phenotype more prevalent among isolates associated with human disease than shed by healthy cattle. The variation consequence heterogeneous expression, being dependent on proportion bacteria population are...
BACKGROUND: Four recent cases of transfusion‐related transmission variant Creutzfeldt‐Jakob disease (vCJD) highlight the need to develop a highly sensitive and specific screening test detect infectivity in blood asymptomatic infected individuals. Protein misfolding cyclic amplification (PMCA), method for minute amounts disease‐associated abnormal prion protein (PrP Sc ) readily detectable levels, could be incorporated into such provided that suitable substrate source routine use human PMCA...
Human prion diseases are a group of rare fatal neurodegenerative conditions with well-developed clinical and neuropathological diagnostic criteria. Recent observations have expanded the spectrum beyond classically recognized forms.In present study we report six patients novel, apparently sporadic disease characterised by thalamic degeneration rapidly progressive dementia (duration illness 2-12 months; age at death: 55-81 years). Light electron microscopic immunostaining for protein (PrP)...
Abstract Human prion diseases, including Creutzfeldt‐Jakob disease (CJD), occur in sporadic, genetic, and acquired forms. Variant (vCJD) first reported 1996 the United Kingdom (UK), resulted from contamination of food with bovine spongiform encephalopathy. There is a concern that UK national surveillance mechanisms might miss some CJD cases (including vCJD), particularly older population where other neurodegenerative disorders are more prevalent. We developed highly sensitive protocol for...
<h3>Introduction</h3> Sporadic CJD is a neurodegenerative illness first described in the 1920s. The condition postulated to be spread by process of direct protein transmission. prion hypothesis suggests that normal cellular proteins (PrPc) convert an abnormal conformation (PrPSc), which forms template for further misfolding. variable phenotype (sCJD) largely felt depend on combination codon 129 genotype gene (MM, MV or VV) and presence two major conformations protease–resistant (type 1 type...