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    Cholestatic Jaundice Associated with Carnitine Palmitoyltransferase IA Deficiency

    Liver dysfunction usually accompanies metabolic decompensation in fatty acid oxidation disorders, including carnitine palmitoyltransferase (CPT) Ia deficiency. Typically, the liver is enlarged with raised plas...

    A A M Morris, S E Olpin, M J Bennett… in JIMD Reports - Case and Research Reports, … (2013)

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    Article

    Develo** X-ray Computed Tomography to non-invasively image 3-D root systems architecture in soil

    The need to observe roots in their natural undisturbed state within soil, both spatially and temporally, is a challenge that continues to occupy researchers studying the rhizosphere.

    S. J. Mooney, T. P. Pridmore, J. Helliwell, M. J. Bennett in Plant and Soil (2012)

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    Pathophysiology of fatty acid oxidation disorders

    Mitochondrial fatty acid oxidation represents an important pathway for energy generation during periods of increased energy demand such as fasting, febrile illness and muscular exertion. In liver, the primary ...

    M. J. Bennett in Journal of Inherited Metabolic Disease (2010)

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    Article

    Mathematical Modelling of the Aux/IAA Negative Feedback Loop

    The hormone auxin is implicated in regulating a diverse range of developmental processes in plants. Auxin acts in part by inducing the Aux/IAA genes. The associated pathway comprises multiple negative feedback lo...

    A. M. Middleton, J. R. King, M. J. Bennett, M. R. Owen in Bulletin of Mathematical Biology (2010)

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    Longitudinal Assessment of Ovarian Perifollicular and Endometrial Vascularity by Power Doppler Ultrasound in Pregnant and Non-Pregnant Cycles in the IVF Setting

    Purpose: This longitudinal study aimed to compare ovarian perifollicular and endometrial blood flow (PFBF and EBF, respectively) during the follicular phase in pregnant and non-pregnant IVF cycles.

    S. M. Shrestha, M. F. Costello, P. Sjoblom… in Journal of Assisted Reproduction and Genet… (2004)

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    Enhanced systemic transgene expression after nonviral salivary gland transfection using a novel endonuclease inhibitor/DNA formulation

    Gene transfer to the major salivary glands is an attractive method for the systemic delivery of therapeutic proteins. To date, nonviral gene transfer to these glands has resulted in inadequate systemic protein...

    E J Niedzinski, Y-J Chen, D C Olson, E A Parker, H Park, J A Udove… in Gene Therapy (2003)

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    Heavy ion collisions at collider energies—Insights from PHENIX

    A. Drees, K. Adcox, S. S. Adler, N. N. Ajitanand, Y. Akiba, J. Alexander… in Pramana (2003)

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    First results from RHIC-PHENIX

    The PHENIX experiment consists of a large detector system located at the newly commissioned relativistic heavy ion collider (RHIC) at the Brookhaven National Laboratory. The primary goal of the PHENIX experime...

    Tarun Kanti Ghosh, K Adcox, S S Adler, N Ajitanand, Y Akiba, J Alexander… in Pramana (2001)

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    Quantitative measurement of total and free 3-hydroxy fatty acids in serum or plasma samples: short-chain 3-hydroxy fatty acids are not esterified

    Diagnostic protocols for disorders of mitochondrial fatty acid oxidation (FAO) generally include the measurement of plasma acylcarnitines. Many biochemical intermediates of FAO resulting from a metabolic block...

    P. M. Jones, A. B. Burlina, M. J. Bennett in Journal of Inherited Metabolic Disease (2000)

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    Acylcarnitines in fibroblasts of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders

    Mitochondrial fatty acid oxidation disorders cause hypoglycaemia, hepatic dysfunction, myopathy, cardiomyopathy and encephalopathy. Despite their recognition for more than 15 years, diagnosis and treatment rem...

    J. J. Shen, D. Matern, D. S. Millington… in Journal of Inherited Metabolic Disease (2000)

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    The neuronal ceroid-lipofuscinoses (Batten disease): A new class of lysosomal storage diseases

    The neuronal ceroid-lipofuscinoses (Batten disease) are a group of severe neurodegenerative disorders characterized clinically by visual loss, seizures and psychomotor degeneration, and pathologically by loss ...

    M. J. Bennett, S. L. Hofmann in Journal of Inherited Metabolic Disease (1999)

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    Article

    N-acetylaspartylglutamate in Canavan disease: an adverse effector?

    We measured N-acetylaspartate and its precursor/product N-acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 3...

    A. P. Burlina, V. Ferrari, P. Divry, W. Gradowska… in European Journal of Pediatrics (1999)

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    Profound neurological phenotype in a patient presenting with disordered isoleucine and energy metabolism

    A. B. Burlina, K. M. Gibson, W. Ruitenbeek… in Journal of Inherited Metabolic Disease (1998)

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    Hypoketonuric 3-hydroxydicarboxylic aciduria in five patients with glycogen storage disease

    C. E. Mize, L. J. Waber, T. Anderson… in Journal of Inherited Metabolic Disease (1997)

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    Polyunsaturated fatty acids reverse the lysosomal storage and accumulation of subunit 9 of mitochondrial F1F0-ATP synthase in cultured lymphoblasts from patients with Batten disease

    M. J. Bennett, R. L. Boriack, R.-M. Boustany in Journal of Inherited Metabolic Disease (1997)

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    Late-onset holocarboxylase synthetase deficiency

    We report a 21-month-old female patient whose urine organic acid profile suggested a biotin utilization abnormality consistent with multiple carboxylase deficiency. For most previously reported patients, holoc...

    K. M. Gibson, M. J. Bennett, W. L. Nyhan… in Journal of Inherited Metabolic Disease (1996)

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    β-Ketothiolase (2-methylacetoacetyl-coenzyme a thiolase) deficiency: identification of two patients in israel

    K. M. Gibson, O. N. Elpeleg, M. J. Bennett in Journal of Inherited Metabolic Disease (1996)

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    Assignment of the human peroxisomal palmitoyl-CoA oxidase gene to chromosome 17q23-qter by PCR technique

    Human peroxisomal palmitoyl-CoA oxidase plays a pivotal role in the β-oxidation of fatty acids. Its importance is reflected by the severity of the disease associated with its deficiency in man. The gene was pr...

    N. N. Moghrabi, D. B. Dawson, M. J. Bennett in Journal of Inherited Metabolic Disease (1995)

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    Medium-chain acyl-CoA dehydrogenase deficiency presenting in the neonatal period: The first Italian case

    A. B. Burlina, M. J. Bennett, N. Gregersen… in European Journal of Pediatrics (1995)

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    Secondary 3-hydroxydicarboxylic aciduria mimicking long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

    M. J. Bennett, M. J. Weinberger, W. G. Sherwood… in Journal of Inherited Metabolic Disease (1994)

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