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N-Acetyl-L-tyrosine is an amino acid, an N-acetyl derivative of tyrosine. It is used for parenteral nutrition and as a dietary supplement. [1] [2] [3] See also.
The liver is the organ affected most by Tyrosinemia Type I due to the high level of expression of the gene for fumarylacetoacetate hydrolase (FAH) in liver cells. The production of blood coagulation factors by the liver is disrupted, causing hemophiliac-like symptoms. Acute liver failure is common, especially in early life.
It is a 4-hydroxyphenylpyruvate dioxygenase inhibitor indicated for the treatment of hereditary tyrosinemia type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine. [7] Liver transplant is indicated for patients with tyrosinemia type I who do not respond to nitisinone, as well as those with acute liver failure and ...
A liver support system or diachysis is a type of therapeutic device to assist in performing the functions of the liver. Such systems focus either on removing the accumulating toxins (liver dialysis), or providing additional replacement of the metabolic functions of the liver through the inclusion of hepatocytes to the device (bioartificial liver device).
Damage to the liver is not due to the drug itself but to a toxic metabolite (N-acetyl-p-benzoquinone imine (NAPQI)) produced by cytochrome P-450 enzymes in the liver. [13] In normal circumstances, this metabolite is detoxified by conjugating with glutathione in phase 2 reaction. In an overdose, a large amount of NAPQI is generated, which ...
The disease results from a deficiency in hepatic tyrosine aminotransferase. [10] Tyrosinemia type II (Richner-Hanhart syndrome, RHS) is a disease of autosomal recessive inheritance characterized by keratitis, palmoplantar hyperkeratosis, mental retardation, and elevated blood tyrosine levels. [10] Keratitis in Tyrosinemia type II patients is ...
N-acetyltransferase (NAT) is an enzyme that catalyzes the transfer of acetyl groups from acetyl-CoA to arylamines, arylhydroxylamines and arylhydrazines. [ 1 ] [ 2 ] [ 3 ] They have wide specificity for aromatic amines , particularly serotonin , and can also catalyze acetyl transfer between arylamines without CoA.
N-acyl amides are a general class of endogenous fatty acid compounds characterized by a fatty acyl group linked to a primary amine metabolite by an amide bond. Broadly speaking, N-acyl amides fall into several categories: amino acid conjugates (e.g., N-arachidonoyl-glycine), neurotransmitter conjugates (e.g., N-arachidonoyl-serotonin), ethanolamine conjugates (e.g., anandamide), and taurine ...