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==Implicatons==

Hyperosmolar hyperglycemic state (HHS) was one of the first diseases that clearly linked GDH regulation to insulin and ammonia homeostasis. Studies demonstrate that the activation of GDH was tightly correlated with increased glutaminolysis and release of insulin. HHS syndrome is caused by the loss of GTP regulation of GDH. Children with HHS have increased β-cell responsiveness to leucine and susceptibility to hypoglycemia following high protein meals. This is due to uncontrolled catabolism of amino acids yielding high ATP levels that stimulate insulin secretion and high serum levels of ammonium. The elevation of serum ammonia levels induces an altered regulation of GDH, leading to increased ammonia production from glutamate oxidation. In addition to that, it can cause impaired urea synthesis by carbmoylphosphate synthetase (CPS) due to reduced formation of N-actyl-glutamate (activator) from glutamate (figure 4). This genetic lesion disrupts the regulator linkage between glycolysis and amino acid catabolism.

[[Image:Example.jpg]]Hyperosmolar hyperglycemic state (HHS) was one of the first diseases that clearly linked GDH regulation to insulin and ammonia homeostasis. Studies demonstrate that the activation of GDH was tightly correlated with increased glutaminolysis and release of insulin. HHS syndrome is caused by the loss of GTP regulation of GDH. Children with HHS have increased β-cell responsiveness to leucine and susceptibility to hypoglycemia following high protein meals. This is due to uncontrolled catabolism of amino acids yielding high ATP levels that stimulate insulin secretion and high serum levels of ammonium. The elevation of serum ammonia levels induces an altered regulation of GDH, leading to increased ammonia production from glutamate oxidation. In addition to that, it can cause impaired urea synthesis by carbmoylphosphate synthetase (CPS) due to reduced formation of N-actyl-glutamate (activator) from glutamate (figure 4). This genetic lesion disrupts the regulator linkage between glycolysis and amino acid catabolism.

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 ==Implicatons==
-Hyperosmolar hyperglycemic state (HHS) was one of the first diseases that clearly linked GDH regulation to insulin and ammonia homeostasis. Studies demonstrate that the activation of GDH was tightly correlated with increased glutaminolysis and release of insulin. HHS syndrome is caused by the loss of GTP regulation of GDH. Children with HHS have increased β-cell responsiveness to leucine and susceptibility to hypoglycemia following high protein meals. This is due to uncontrolled catabolism of amino acids yielding high ATP levels that stimulate insulin secretion and high serum levels of ammonium. The elevation of serum ammonia levels induces an altered regulation of GDH, leading to increased ammonia production from glutamate oxidation. In addition to that, it can cause impaired urea synthesis by carbmoylphosphate synthetase (CPS) due to reduced formation of N-actyl-glutamate (activator) from glutamate (figure 4). This genetic lesion disrupts the regulator linkage between glycolysis and amino acid catabolism.
+[[Image:Example.jpg]]Hyperosmolar hyperglycemic state (HHS) was one of the first diseases that clearly linked GDH regulation to insulin and ammonia homeostasis. Studies demonstrate that the activation of GDH was tightly correlated with increased glutaminolysis and release of insulin. HHS syndrome is caused by the loss of GTP regulation of GDH. Children with HHS have increased β-cell responsiveness to leucine and susceptibility to hypoglycemia following high protein meals. This is due to uncontrolled catabolism of amino acids yielding high ATP levels that stimulate insulin secretion and high serum levels of ammonium. The elevation of serum ammonia levels induces an altered regulation of GDH, leading to increased ammonia production from glutamate oxidation. In addition to that, it can cause impaired urea synthesis by carbmoylphosphate synthetase (CPS) due to reduced formation of N-actyl-glutamate (activator) from glutamate (figure 4). This genetic lesion disrupts the regulator linkage between glycolysis and amino acid catabolism.
 ==Applications==