The Experts below are selected from a list of 249 Experts worldwide ranked by ideXlab platform
M Rashed - One of the best experts on this subject based on the ideXlab platform.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism.
Brain & development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1-5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10-25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with beta-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate carboxylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocarboxylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism
Brain and Development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1–5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10–25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with β-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate car☐ylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocar☐ylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
H G Worthen - One of the best experts on this subject based on the ideXlab platform.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism.
Brain & development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1-5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10-25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with beta-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate carboxylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocarboxylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism
Brain and Development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1–5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10–25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with β-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate car☐ylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocar☐ylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
Miguel A Lanaspa - One of the best experts on this subject based on the ideXlab platform.
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Cerebral Fructose Metabolism as a Potential Mechanism Driving Alzheimer's Disease.
Frontiers in aging neuroscience, 2020Co-Authors: Richard J. Johnson, Dean R Tolan, Fernando Gomez-pinilla, Maria A. Nagel, Takahiko Nakagawa, Bernardo Rodriguez-iturbe, Laura G. Sánchez-lozada, Miguel A LanaspaAbstract:The loss of cognitive function in Alzheimer's disease is pathologically linked with neurofibrillary tangles, amyloid deposition, and loss of neuronal communication. Cerebral insulin resistance and mitochondrial dysfunction have emerged as important contributors to pathogenesis supporting our hypothesis that cerebral Fructose Metabolism is a key initiating pathway for Alzheimer's disease. Fructose is unique among nutrients because it activates a survival pathway to protect animals from starvation by lowering energy in cells in association with adenosine monophosphate degradation to uric acid. The fall in energy from Fructose Metabolism stimulates foraging and food intake while reducing energy and oxygen needs by decreasing mitochondrial function, stimulating glycolysis, and inducing insulin resistance. When Fructose Metabolism is overactivated systemically, such as from excessive Fructose intake, this can lead to obesity and diabetes. Herein, we present evidence that Alzheimer's disease may be driven by overactivation of cerebral Fructose Metabolism, in which the source of Fructose is largely from endogenous production in the brain. Thus, the reduction in mitochondrial energy production is hampered by neuronal glycolysis that is inadequate, resulting in progressive loss of cerebral energy levels required for neurons to remain functional and viable. In essence, we propose that Alzheimer's disease is a modern disease driven by changes in dietary lifestyle in which Fructose can disrupt cerebral Metabolism and neuronal function. Inhibition of intracerebral Fructose Metabolism could provide a novel way to prevent and treat this disease.
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Fructose contributes to the Warburg effect for cancer growth.
Cancer & metabolism, 2020Co-Authors: Takahiko Nakagawa, Dean R Tolan, Christopher J Rivard, Miguel A Lanaspa, Laura G. Sánchez-lozada, Ana Andres-hernando, Inigo San Millan, Mehdi A. Fini, Richard J. JohnsonAbstract:Obesity and metabolic syndrome are strongly associated with cancer, and these disorders may share a common mechanism. Recently, Fructose has emerged as a driving force to develop obesity and metabolic syndrome. Thus, we assume that Fructose may be the mechanism to explain why obesity and metabolic syndrome are linked with cancer. Clinical and experimental evidence showed that Fructose intake was associated with cancer growth and that Fructose transporters are upregulated in various malignant tumors. Interestingly, Fructose Metabolism can be driven under low oxygen conditions, accelerates glucose utilization, and exhibits distinct effects as compared to glucose, including production of uric acid and lactate as major byproducts. Fructose promotes the Warburg effect to preferentially downregulate mitochondrial respiration and increases aerobic glycolysis that may aid metastases that initially have low oxygen supply. In the process, uric acid may facilitate carcinogenesis by inhibiting the TCA cycle, stimulating cell proliferation by mitochondrial ROS, and blocking fatty acid oxidation. Lactate may also contribute to cancer growth by suppressing fat oxidation and inducing oncogene expression. The ability of Fructose Metabolism to directly stimulate the glycolytic pathway may have been protective for animals living with limited access to oxygen, but may be deleterious toward stimulating cancer growth and metastasis for humans in modern society. Blocking Fructose Metabolism may be a novel approach for the prevention and treatment of cancer.
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deletion of fructokinase in the liver or in the intestine reveals differential effects on sugar induced metabolic dysfunction
Cell Metabolism, 2020Co-Authors: Ana Andreshernando, Takuji Ishimoto, Richard J. Johnson, Takahiko Nakagawa, David J Orlicky, Masanari Kuwabara, Miguel A LanaspaAbstract:Intake of Fructose-containing sugars is strongly associated with metabolic syndrome. Compared with other sugars, dietary Fructose is uniquely metabolized by fructokinase. However, the tissue-specific role of fructokinase in sugar-induced metabolic syndrome, and the specific roles of glucose and Fructose in driving it, is not fully understood. Here, we show that in mice receiving excess Fructose-glucose solutions, whole-body deletion of fructokinase, and thus full blockade of Fructose Metabolism, is sufficient to prevent metabolic syndrome. This protection is not only due to reduced Fructose Metabolism, but also due to decreased sugar intake. Furthermore, by using tissue-specific fructokinase-deficient mice, we determined that while sugar intake is controlled by intestinal fructokinase activity, metabolic syndrome is driven by Fructose Metabolism in the liver. Our findings show a two-pronged role for Fructose Metabolism in sugar-induced metabolic syndrome, one arm via the intestine that mediates sugar intake and a second arm in the liver that drives metabolic dysfunction.
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Fructose Production and Metabolism in the Kidney.
Journal of the American Society of Nephrology : JASN, 2020Co-Authors: Takahiko Nakagawa, Dean R Tolan, Richard J. Johnson, Laura G. Sánchez-lozada, Ana Andres-hernando, Carlos A. Roncal-jimenez, Miguel A LanaspaAbstract:Understanding Fructose Metabolism might provide insights to renal pathophysiology. To support systemic glucose concentration, the proximal tubular cells reabsorb Fructose as a substrate for gluconeogenesis. However, in instances when Fructose intake is excessive, Fructose Metabolism is costly, resulting in energy depletion, uric acid generation, inflammation, and fibrosis in the kidney. A recent scientific advance is the discovery that Fructose can be endogenously produced from glucose under pathologic conditions, not only in kidney diseases, but also in diabetes, in cardiac hypertrophy, and with dehydration. Why humans have such a deleterious mechanism to produce Fructose is unknown, but it may relate to an evolutionary benefit in the past. In this article, we aim to illuminate the roles of Fructose as it relates to gluconeogenesis and fructoneogenesis in the kidney.
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Fructose Metabolism and downstream effects.
2016Co-Authors: Miguel A Lanaspa, Christina Cicerchi, Christopher J Rivard, Keith R. Randolph, Jatinder Rana, Jeffrey D. Scholten, Brandi L. Hunter, Richard J. JohnsonAbstract:ALDOB aldolase B, AMPD2 adenosine monophosphate deaminase 2, AMPK AMP-activated protein kinase, DAK dihydroxyacetone kinase, FAS fatty acid synthase, KHK ketohexokinase.
A Al Odaib - One of the best experts on this subject based on the ideXlab platform.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism.
Brain & development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1-5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10-25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with beta-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate carboxylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocarboxylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism
Brain and Development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1–5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10–25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with β-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate car☐ylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocar☐ylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
S B Subramanyam - One of the best experts on this subject based on the ideXlab platform.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism.
Brain & development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1-5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10-25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with beta-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate carboxylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocarboxylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
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Comparative frequency and severity of hypoglycemia in selected organic acidemias, branched chain amino acidemia, and disorders of Fructose Metabolism
Brain and Development, 1994Co-Authors: H G Worthen, A Al Ashwal, P T Ozand, S Garawi, Z Rahbeeni, A Al Odaib, S B Subramanyam, M RashedAbstract:The Institution's experience with hypoglycemia in different types of organic acidemias, branched chain amino acidemia (MSUD), and disorders of Fructose Metabolism was reviewed retrospectively. The charts of 144 patients who were followed for 1–5 years were studied for the severity and frequency of hypoglycemia. The patients were mainly Saudi; however, 10–25% were from neighboring countries. Therefore, the observations pertain to the genetic groups in the Arabian peninsula. Organic acidemias which primarily manifest with neurologic signs, such as 4-hydroxybutyric aciduria, infantile onset 3-methylglutaconic aciduria, and glutaric aciduria type 1 never showed hypoglycemia. Patients with β-ketothiolase deficiency, biotinidase deficiency, or intermittent or intermediate MSUD, also did not have hypoglycemia during metabolic crisis. Hypoglycemia was rare and mild among neonates with classic MSUD, ethylmalonic aciduria, and isovaleric acidemia. Less than 50% of the patients with MSUD older than 8 months, pyruvate car☐ylase deficiency, methylmalonic acidemia, or propionic acidemia had hypoglycemia during metabolic crisis. On the other hand, patients with 3-hydroxy-3-methyl glutaryl-CoA lyase deficiency, holocar☐ylase synthetase deficiency, medium or long-chain acyl-CoA dehydrogenase deficiency, neonatal onset 3-methylglutaconic aciduria, glutaric aciduria type 2, and disorders of Fructose Metabolism invariably had moderate-to-severe hypoglycemia associated with metabolic crisis. The purpose of this report is to provide the pediatrician, particularly in the Middle East, with a diagnostic guideline to the identification and management of different types of organic acidemias, based on co-existing hypoglycemia.
