Search Results - "Paul, Harbhajan S."

Production and characterization of murine models of classic and intermediate maple syrup urine disease by Homanics, Gregg E, Skvorak, Kristen, Ferguson, Carolyn, Watkins, Simon, Paul, Harbhajan S

“…Maple Syrup Urine Disease (MSUD) is an inborn error of metabolism caused by a deficiency of branched-chain keto acid dehydrogenase. MSUD has several clinical…”
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Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease by Zinnanti, William J., Lazovic, Jelena, Griffin, Kathleen, Skvorak, Kristen J., Paul, Harbhajan S., Homanics, Gregg E., Bewley, Maria C., Cheng, Keith C., LaNoue, Kathryn F., Flanagan, John M.

“…Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with life-threatening cerebral oedema and…”
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Hepatocyte Transplantation Improves Phenotype and Extends Survival in a Murine Model of Intermediate Maple Syrup Urine Disease by Skvorak, Kristen J, Paul, Harbhajan S, Dorko, Kenneth, Marongiu, Fabio, Ellis, Ewa, Chace, Donald, Ferguson, Carolyn, Gibson, K Michael, Homanics, Gregg E, Strom, Stephen C

“…Maple syrup urine disease (MSUD; OMIM 248600) is an inborn error of metabolism of the branched chain α-ketoacid dehydrogenase (BCKDH) complex that is treated…”
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Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD) by Skvorak, Kristen J., Hager, Elizabeth J., Arning, Erland, Bottiglieri, Teodoro, Paul, Harbhajan S., Strom, Stephen C., Homanics, Gregg E., Sun, Qin, Jansen, Erwin E.W., Jakobs, Cornelis, Zinnanti, William J., Gibson, K. Michael

“…Skvorak et al. [1] demonstrated the therapeutic efficacy of HTx in a murine model of iMSUD, confirming significant metabolic improvement and survival. To…”
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Inverse alterations of BCKA dehydrogenase activity in cardiac and skeletal muscles of diabetic rats by Lombardo, Y.B, Serdikoff, C, Thamotharan, M, Paul, H.S, Adibi, S.A

“…Rat cardiac and skeletal muscles, which have been used as model tissues for studies of regulation of branched-chain alpha-keto acid (BCKA) oxidation, vary…”
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Hyperammonemia and carnitine deficiency in a patient receiving sulfadiazine and pyrimethamine by Sekas, G, Paul, H S

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Production and Characterization of Maple Syrup Urine Disease Murine Models by Skvorak, Kristen J, Paul, Harbhajan S, Ferguson, Carolyn, Watkins, Simon, Homanics, Gregg E

“…Maple Syrup Urine Disease (MSUD) is a genetic disorder caused by deficiency of branched‐chain á‐keto acid dehydrogenase (BCKDH) and results in neurological…”
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Paradoxical Effects of Clofibrate on Liver and Muscle Metabolism in Rats by Paul, Harbhajan S., Adibi, Siamak A.

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Involvement of transverse tubules in induced myotonia by Ontell, M, Paul, H S, Adibi, S A, Martin, J L

“…A single oral dose of clofibrate (30 mg/100 gm body weight) given to 250-300 gm male rats produced myotonic discharges from both the gastrocnemius and soleus…”
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Alteration in gene expression of branched-chain keto acid dehydrogenase kinase but not in gene expression of its substrate in the liver of clofibrate-treated rats by Paul, H S, Liu, W Q, Adibi, S A

“…We previously showed that the oxidation of branched-chain amino acids is increased in rats treated with clofibrate [Paul and Adibi (1980) J. Clin. Invest. 65,…”
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Regulation of gene expression of branched-chain keto acid dehydrogenase complex in primary cultured hepatocytes by dexamethasone and a cAMP analog by A G Chicco, S A Adibi, W Q Liu, S M Morris, Jr, H S Paul

“…The present study demonstrates that dexamethasone and 8-(4-chlorophenylthio)adenosine 3',5'-monophosphate (CPT-cAMP), a cAMP analog, increase the substrate…”
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Mechanism of increased conversion of branched chain keto acid dehydrogenase from inactive to active form by a medium chain fatty acid (octanoate) in skeletal muscle by PAUL, H. S, ADIBI, S. A

“…We and others have previously shown that octanoate increases the oxidation of branched chain amino acids (BCAA) in skeletal muscle. The present study was…”
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Role of testosterone in the induction of hepatic peroxisome proliferation by clofibrate by Paul, H S, Sekas, G, Winters, S J

“…Hepatic peroxisome proliferation is induced by a number of agents, including clofibrate. Sustained proliferation of peroxisomes is associated with the…”
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Inhibition of carnitine acetyltransferase by bile acids: implications for carnitine analysis by Sekas, G, Paul, H S

“…Carnitine acetyltransferase is used in a radioenzymatic assay to measure the concentration of carnitine. While determining the concentration of carnitine in…”
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Carnitine biosynthesis in hepatic peroxisomes by PAUL, Harbhajan S., SEKAS, Gail, ADIBI, Siamak A.

“…We have investigated whether hepatic peroxisomes are capable of synthesizing carnitine. When purified peroxisomes were incubated with γ‐butyrobetaine, a…”
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Systemic Carnitine Deficiency: A Treatable Disorder by Brass, Eric P., Paul, Harbhajan S., Sekas, Gail

“…A 3.5-year-old boy presented to the emergency room with hypoglycemia. The patient’s history was remarkable for multiple episodes of altered consciousness…”
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Paradoxical effects of clofibrate on liver and muscle metabolism in rats. Induction of myotonia and alteration of fatty acid and glucose oxidation by Paul, H S, Adibi, S A

“…Chronic clofibrate intake, on occasion, results in a muscular syndrome in man. We have investigated the effects of chronic clofibrate administration in rats on…”
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Carnitine biosynthesis in hepatic peroxisomes : demonstration of γ-butyrobetaine hydroxylase activity by PAUL, H. S, SEKAS, G, ADIBI, S. A

“…We have investigated whether hepatic peroxisomes are capable of synthesizing carnitine. When purified peroxisomes were incubated with gamma-butyrobetaine, a…”
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Leucine oxidation and protein turnover in clofibrate-induced muscle protein degradation in rats by Paul, H S, Adibi, S A

“…Treatment of hyperlipidemia with clofibrate may result in development of a muscular syndrome. Our previous investigation (1979. J. Clin. Invest.64: 405.)…”
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Inhibition of carnitine acyltransferase activities by bile acids in rat liver peroxisomes by Sekas, G, Paul, H S

“…We recently reported that purified carnitine acetyltransferase is competitively inhibited by bile acids (Sekas, G. and Paul, H.S. (1989) Anal. Biochem. 179,…”
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