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Suzanne M. K. Buckley
University College London
127Publications
26H-index
2,413Citations
Publications 127
Newest
#1N Suff (UCL: University College London)H-Index: 3
#2Rajvinder Karda (UCL: University College London)H-Index: 5
Last.Donald Peebles (UCL: University College London)H-Index: 31
view all 12 authors...
Approximately 40% of preterm births are preceded by microbial invasion of the intrauterine space: ascent from the vagina is the most common pathway. Within the cervical canal, antimicrobial peptides and proteins (AMPs) help to constitute a barrier which prevents ascending infection. We investigated whether expression of the AMP, human β-defensin-3 (HBD3), in the cervical mucosa prevented bacterial ascent from the vagina into the uterine cavity of pregnant mice. An adeno-associated virus vector c...
#1Julien Baruteau (UCL: University College London)H-Index: 7
#2Dany P. Perocheau (UCL: University College London)H-Index: 5
Last.Simon N. Waddington (UCL: University College London)H-Index: 40
view all 22 authors...
Argininosuccinate lyase (ASL) belongs to the hepatic urea cycle detoxifying ammonia, and the citrulline-nitric oxide (NO) cycle producing NO. ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia, multiorgan disease and neurocognitive impairment despite treatment aiming to normalise ammonaemia without considering NO imbalance. Here we show that cerebral disease in argininosuccinic aciduria involves neuronal oxidative/nitrosative stress independent of hyperammo...
#1N Suff (UCL: University College London)H-Index: 3
#2Rajvinder Karda (UCL: University College London)H-Index: 5
Last.Simon N. Waddington (UCL: University College London)H-Index: 40
view all 11 authors...
Preterm birth is a serious global health problem and the leading cause of infant death before 5 years of age. At least 40% of cases are associated with infection. The most common way for pathogens to access the uterine cavity is by ascending from the vagina. Bioluminescent pathogens have revolutionized the understanding of infectious diseases. We hypothesized that bioluminescent Escherichia coli can be used to track and monitor ascending vaginal infections. Two bioluminescent strains were studie...
#1John R. Counsell (UCL: University College London)H-Index: 4
#2Rajvinder Karda (UCL: University College London)H-Index: 5
Last.Steven J. Howe (UCL: University College London)H-Index: 25
view all 19 authors...
Viral vectors are rapidly being developed for a range of applications in research and gene therapy. Prototype foamy virus (PFV) vectors have been described for gene therapy, although their use has mainly been restricted to ex vivo stem cell modification. Here we report direct in vivo transgene delivery with PFV vectors carrying reporter gene constructs. In our investigations, systemic PFV vector delivery to neonatal mice gave transgene expression in the heart, xiphisternum, liver, pancreas, and ...
#1Giulia Massaro (UCL: University College London)H-Index: 4
#2Citra Nurfarah Zaini Mattar (NUS: National University of Singapore)H-Index: 13
Last.Ahad A. Rahim (UCL: University College London)H-Index: 18
view all 22 authors...
For inherited genetic diseases, fetal gene therapy offers the potential of prophylaxis against early, irreversible and lethal pathological change. To explore this, we studied neuronopathic Gaucher disease (nGD), caused by mutations in GBA. In adult patients, the milder form presents with hepatomegaly, splenomegaly and occasional lung and bone disease; this is managed, symptomatically, by enzyme replacement therapy. The acute childhood lethal form of nGD is untreatable since enzyme cannot cross t...
#1Julien Baruteau (UCL: University College London)H-Index: 7
#2Dany P. Perocheau (UCL: University College London)H-Index: 5
Last.Simon N. Waddington (UCL: University College London)H-Index: 40
view all 20 authors...
Argininosuccinate lyase (ASL) belongs to the liver-based urea cycle detoxifying ammonia, and the citrulline-nitric oxide cycle synthesising nitric oxide (NO). ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia and a multi-organ disease with neurocognitive impairment. Current therapeutic guidelines aim to control ammonaemia without considering the systemic NO imbalance. Here, we observed a neuronal disease with oxidative/nitrosative stress in ASL-deficient m...
#1Niraja Dighe (NUS: National University of Singapore)H-Index: 5
#2Kang Wei Tan (NUS: National University of Singapore)
Last.Citra Nurfarah Zaini Mattar (NUS: National University of Singapore)H-Index: 13
view all 14 authors...
Major hemoglobinopathies place tremendous strain on global resources. Intrauterine hemopoietic cell transplantation (IUHCT) and gene transfer (IUGT) can potentially reduce perinatal morbidities with greater efficacy than postnatal therapy alone. We performed both procedures in the thalassemic HbbTh3/+ mouse. Intraperitoneal delivery of co-isogenic cells at embryonic days13-14 produced dose-dependent chimerism. High-dose adult bone marrow (BM) cells maintained 0.2–3.1% chimerism over ~24 weeks an...
#1Rajvinder Karda (UCL: University College London)H-Index: 5
#2Ahad A. Rahim (UCL: University College London)H-Index: 18
Last.Simon N. Waddington (UCL: University College London)H-Index: 40
view all 15 authors...
We have previously designed a library of lentiviral vectors to generate somatic-transgenic rodents to monitor signalling pathways in diseased organs using whole-body bioluminescence imaging, in conscious, freely moving rodents. We have now expanded this technology to adeno-associated viral vectors. We first explored bio-distribution by assessing GFP expression after neonatal intravenous delivery of AAV8. We observed widespread gene expression in, central and peripheral nervous system, liver, kid...
#1Rajvinder Karda (UCL: University College London)H-Index: 5
#2Dany P. Perocheau (UCL: University College London)H-Index: 5
Last.Simon N. Waddington (UCL: University College London)H-Index: 40
view all 12 authors...
Luciferase bioimaging in living animals is increasingly being applied in many fields of biomedical research. Rodent imaging usually involves anaesthetising the animal during data capture, however, the biological consequences of anaesthesia have been largely overlooked. We have evaluated luciferase bioimaging in conscious, unrestrained mice after neonatal intracranial or intravascular administration of lentiviral, luciferase reporter cassettes (biosensors); we present real-time analyses from the ...
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