Search Results - "Hemsley, M."
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Endo‐lysosomal and autophagic dysfunction: a driving factor in Alzheimer's disease?
Published in Journal of neurochemistry (01-03-2017)“…Alzheimer's disease (AD) is the most common cause of dementia, and its prevalence will increase significantly in the coming decades. Although important…”
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The HicA toxin from Burkholderia pseudomallei has a role in persister cell formation
Published in Biochemical journal (15-04-2014)“…TA (toxin-antitoxin) systems are widely distributed amongst bacteria and are associated with the formation of antibiotic tolerant (persister) cells that may…”
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3
Extensive genome analysis of Coxiella burnetii reveals limited evolution within genomic groups
Published in BMC genomics (05-06-2019)“…Coxiella burnetii is a zoonotic pathogen that resides in wild and domesticated animals across the globe and causes a febrile illness, Q fever, in humans. An…”
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Burkholderia thailandensis strain E555 is a surrogate for the investigation of Burkholderia pseudomallei replication and survival in macrophages
Published in BMC microbiology (15-05-2019)“…Burkholderia pseudomallei is a human pathogen causing severe infections in tropical and subtropical regions and is classified as a bio-threat agent. B…”
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A Preclinical Study Evaluating AAVrh10-Based Gene Therapy for Sanfilippo Syndrome
Published in Human gene therapy (01-05-2016)“…Mucopolysaccharidosis type IIIA (MPS IIIA) is predominantly a disorder of the central nervous system, caused by a deficiency of sulfamidase (SGSH) with…”
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Compromised transcription-mRNA export factor THOC2 causes R-loop accumulation, DNA damage and adverse neurodevelopment
Published in Nature communications (08-02-2024)“…We implicated the X-chromosome THOC2 gene, which encodes the largest subunit of the highly-conserved TREX ( Tr anscription- Ex port) complex, in a clinically…”
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Correlating Genotyping Data of Coxiella burnetii with Genomic Groups
Published in Pathogens (Basel) (14-05-2021)“…Coxiella burnetii is a zoonotic pathogen that resides in wild and domesticated animals across the globe and causes a febrile illness, Q fever, in humans…”
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8
Progress in antischistosomal N,N′-diaryl urea SAR
Published in Bioorganic & medicinal chemistry letters (01-02-2018)“…[Display omitted] •Expansion of antischistosomal N,N′-diaryl urea SAR.•3-Trifluoromethyl-4-pyridyl and 2,2-difluorobenzodioxole improve…”
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Genome-wide saturation mutagenesis of Burkholderia pseudomallei K96243 predicts essential genes and novel targets for antimicrobial development
Published in mBio (11-02-2014)“…Burkholderia pseudomallei is the causative agent of melioidosis, an often fatal infectious disease for which there is no vaccine. B. pseudomallei is listed as…”
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Identification of Burkholderia pseudomallei Genes Induced During Infection of Macrophages by Differential Fluorescence Induction
Published in Frontiers in microbiology (21-02-2020)“…, the causative agent of melioidosis, can survive and replicate in macrophages. Little is known about genes that are induced during macrophage infection. We…”
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AAVrh10 Vector Corrects Disease Pathology in MPS IIIA Mice and Achieves Widespread Distribution of SGSH in Large Animal Brains
Published in Molecular therapy. Methods & clinical development (12-06-2020)“…Patients with mucopolysaccharidosis type IIIA (MPS IIIA) lack the lysosomal enzyme sulfamidase (SGSH), which is responsible for the degradation of heparan…”
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Determination of the role of injection site on the efficacy of intra-CSF enzyme replacement therapy in MPS IIIA mice
Published in Molecular genetics and metabolism (01-05-2015)“…MPS IIIA is an inherited neurodegenerative lysosomal storage disorder characterized by cognitive impairment, sleep–wake cycle disturbance, speech difficulties,…”
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Reduction in open field activity in the absence of memory deficits in the AppNL−G−F knock-in mouse model of Alzheimer’s disease
Published in Behavioural brain research (15-01-2018)“…•AppNL−G−F mice show reduced open field activity at six months of age.•Detection of memory deficits in six month AppNL−G−F mice may depend on…”
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14
Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice
Published in Behavioural brain research (05-08-2008)“…Mucopolysaccharidosis (MPS) IIIA, or Sanfilippo syndrome, is a lysosomal storage disorder characterized by severe and progressive neuropathology. Following an…”
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15
Enzyme replacement reduces neuropathology in MPS IIIA dogs
Published in Neurobiology of disease (01-08-2011)“…Abstract There is no treatment for the progressive neurodegenerative lysosomal storage disorder mucopolysaccharidosis type IIIA (MPS IIIA), which occurs due to…”
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16
Is the eye a window to the brain in Sanfilippo syndrome?
Published in Acta neuropathologica communications (17-11-2020)“…Sanfilippo syndrome is an untreatable form of childhood-onset dementia. Whilst several therapeutic strategies are being evaluated in human clinical trials…”
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Examination of intravenous and intra-CSF protein delivery for treatment of neurological disease
Published in The European journal of neuroscience (01-03-2009)“…Mucopolysaccharidosis type IIIA is a neurodegenerative lysosomal storage disorder characterized by progressive loss of learned skills, sleep disturbance and…”
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Lysosomal gene Hexb displays haploinsufficiency in a knock-in mouse model of Alzheimer’s disease
Published in IBRO neuroscience reports (01-06-2022)“…Lysosomal network abnormalities are an increasingly recognised feature of Alzheimer’s disease (AD), which appear early and are progressive in nature. Sandhoff…”
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Evaluating the relationship between acidogenicity and acid tolerance for oral streptococci from children with or without a history of caries
Published in Journal of oral microbiology (01-01-2020)“…Background: Dental caries etiology is attributed to a dysbiotic imbalance within the plaque microbiome leading to a dominance of strong acidogens. Some studies…”
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A lower motor neuron disease in takahē (Porphyrio hochstetteri) is an endoplasmic reticulum storage disease
Published in New Zealand veterinary journal (04-07-2023)“…To investigate the pathogenesis of a disease in takahē (Porphyrio hochstetteri) with intracytoplasmic inclusion bodies in lower motor neurons. Four birds aged…”
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