A5107975034- Metabolism and Genetic Disorders
- Peroxisome Proliferator-Activated Receptors
- Diet and metabolism studies
- Adipose Tissue and Metabolism
- Mitochondrial Function and Pathology
- Historical and Linguistic Studies
- Islamic Studies and History
- Teacher Education and Leadership Studies
- Byzantine Studies and History
- Lysosomal Storage Disorders Research
- Archaeology and Historical Studies
- Animal Genetics and Reproduction
- Higher Education Practises and Engagement
- Evaluation of Teaching Practices
- Reflective Practices in Education
- Virus-based gene therapy research
- Historical, Religious, and Philosophical Studies
- Historical and Religious Studies of Rome
- Lipid metabolism and biosynthesis
- Cancer, Hypoxia, and Metabolism
- Metabolomics and Mass Spectrometry Studies
- Educational Assessment and Improvement
- Education and Islamic Studies
- Muscle metabolism and nutrition
- Higher Education Governance and Development
Nottingham Trent University
2024-2025
Bishop Grosseteste University
2018-2022
Waitemata District Health Board
2022
University of Leicester
2013-2018
St James's University Hospital
2007-2018
Newcastle University
2017
Birmingham City University
2017
Sanford Burnham Prebys Medical Discovery Institute
2009-2013
University of Toronto
2013
University of Alabama at Birmingham
2002-2012
Alterations in mitochondrial function have been implicated the pathogenesis of insulin resistance and type 2 diabetes. However, it is unclear whether reduced a primary or acquired defect this process. To determine defects β-oxidation can cause resistance, we studied mice with deficiency long-chain acyl-CoA dehydrogenase (LCAD), key enzyme fatty acid oxidation. Here, show that LCAD knockout develop hepatic steatosis, which associated as reflected by suppression glucose production during...
Abnormalities of fatty acid metabolism are recognized to play a significant role in human disease, but the mechanisms remain poorly understood. Long-chain acyl-CoA dehydrogenase (LCAD) catalyzes initial step mitochondrial oxidation (FAO). We produced mouse model LCAD deficiency with severely impaired FAO. Matings between +/− mice yielded an abnormally low number and −/− offspring, indicating frequent gestational loss. that reached birth appeared normal, had reduced fasting tolerance hepatic...
Background— Carnitine palmitoyltransferase-1 (CPT1) is a rate-limiting step of mitochondrial β-oxidation by controlling the uptake long-chain acyl-CoAs. The muscle isoform, CPT1b, predominant isoform expressed in heart. It has been suggested that inhibiting CPT1 activity specific inhibitors exerts protective effects against cardiac hypertrophy and heart failure. However, clinical animal studies have shown mixed results, thereby creating concerns about safety this class drugs. Preclinical...
Activated Phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in PIK3CD (APDS1) or PIK3R1 (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims characterize course, identify outcome predictors...
Muscular dystrophies include a diverse group of genetically heterogeneous disorders that together affect 1 in 2000 births worldwide. The diseases are characterized by progressive muscle weakness and wasting lead to severe disability often premature death. Rostrocaudal muscular dystrophy (rmd) is new recessive mouse mutation causes rapidly neonatal forelimb bone deformity. rmd 1.6-kb intragenic deletion within the choline kinase beta (Chkb) gene, resulting complete loss CHKB protein enzymatic...
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inherited disorder of mitochondrial fatty acid beta-oxidation in humans. To better understand pathogenesis this disease, we developed a mouse model for MCAD (MCAD-/-) by gene targeting embryonic stem (ES) cells. The MCAD-/- mice an organic aciduria and liver, showed profound cold intolerance at 4 degrees C with prior fasting. sporadic cardiac lesions seen have not been reported human patients. There was significant...
In this paper, we report on data from our survey of the university Initial Teacher Education (ITE) sector in England, concerning responses to questions about perceptions workload and research. Our collects annually (since 2021) approximately 12% cohort, includes a variety topics, with Likert scale text responses. Here, three interconnected areas potential impacts future teacher education pertinent findings for other nations, including research expectations opportunities, workload, extent...
In the present paper, we describe a novel method which enables analysis of tissue acylcarnitines and carnitine biosynthesis intermediates in same sample. This was used to investigate fatty acid metabolism wild-type LCAD−/− (long-chain acyl-CoA dehydrogenase-deficient) mice. agreement with previous results plasma bile, found accumulation characteristic C14:1-acylcarnitine all investigated tissues from Surprisingly, quantitatively relevant levels 3-hydroxyacylcarnitines were be heart, muscle...
Carnitine palmitoyltransferase-I (CPT-I) catalyzes the rate-controlling step of fatty acid oxidation. CPT-I converts long-chain acyl-CoAs to acylcarnitines for translocation across mitochondrial membrane. The mRNA levels and enzyme activity liver isoform, CPT-Iα, are greatly increased in hyperthyroid animals. Thyroid hormone (T3) stimulates CPT-Iα transcription far more robustly than non-hepatic tissues. We have shown that thyroid receptor (TR) binds a response element (TRE) located...
The genes for apolipoprotein B and microsomal triglyceride transfer protein are expressed in mouse human heart tissue. Why the would express these "lipoprotein assembly" has been unclear. Here we demonstrate that beating actually secretes spherical lipoproteins. Moreover, increased cardiac production of lipoproteins (<i>e.g</i>., mice a transgene) was associated with secretion from decreased stores triglycerides within heart. Increased also reduced pathological accumulation occurs hearts...
Cardiac hypertrophy is a common finding in human patients with inborn errors of long-chain fatty acid oxidation. Mice either very acyl-coenzyme A dehydrogenase deficiency (VLCAD-/-) or (LCAD-/-) develop cardiac hypertrophy. hypertrophy, initially measured using heart/body weight ratios, was manifested most severely LCAD-/- male mice. VLCAD-/- mice, as group, showed mild increase normalized mass (8.8% compared all wild-type (WT) mice). In contrast, mice group more severe (32.2% WT On the...
The efficacy, safety, and metabolic consequences of rapid weight loss in privately owned obese cats by means a canned weightreduction diet the influence orally administered L-carnitine on rate loss, routine clinical evaluations, hepatic ultrasonography, plasma amino acid profiles, carnitine analytes were evaluated.A double-blinded placebo-controlled design was used with randomly divided into 2 groups: Group 1 (n ϭ 14) received (250 mg PO q24h) aqueous solution group 10) an...
Abstract: Neurochemical studies were performed on synaptosomal membranes from cats with GM 1 or 2 gangliosidosis to examine possible mechanisms of neuronal dysfunction in these disorders. The basic hypothesis tested was that deficient ganglioside catabolism causes increased content plasma membrane which turn disrupts normal function. Fluidity characteristics examined using fluorescence polarization. Results showed markedly reduced fluidity both and gangliosidosis. These results supported by...