Proteins aminotransferases

ALT, alanine aminotransferase ASC, apoptosis-associated speck-like protei containing a CARD AST, aspartate aminotransferase CARD, caspase activation and recruitment domains CD, Crohn s disease COP, CARD-only protein DD, death domain DED, death effector domains DIABLO, direct LAP-binding protein with low pi... 

The probable metabohc defect in type I tyrosine-mia (tyrosinosis) is at himarylacetoacetate hydrolase (reaction 4, Figure 30-12). Therapy employs a diet low in tyrosine and phenylalanine. Untreated acute and chronic tyrosinosis leads to death from liver failure. Alternate metabolites of tyrosine are also excreted in type II tyrosinemia (Richner-Hanhart syndrome), a defect in tyrosine aminotransferase (reaction 1, Figure 30-12), and in neonatal tyrosinemia, due to lowered y>-hydroxyphenylpyruvate hydroxylase activity (reaction 2, Figure 30-12). Therapy employs a diet low in protein. 

Pantothenic acid is present in coenzyme A and acyl carrier protein, which act as carriers for acyl groups in metabolic reactions. Pyridoxine, as pyridoxal phosphate, is the coenzyme for several enzymes of amino acid metabolism, including the aminotransferases, and of glycogen phosphorylase. Biotin is the coenzyme for several carboxylase enzymes. 

Arabinose-binding protein domains 1 and 2 Dihydrofolate reductase Adenylate kinase Rhodanese domains 1 and 2 Glutathione reductase domains 1 and 2 Phosphoglycerate mutase Phosphoglycerate kinase domain 2 Pyruvate kinase domain 3 Hexokinase domains 1 and 2 Catalase domain 3 Aspartate aminotransferase... 

Pyridoxine (B ) Pyridoxal-P (PLP) Aminotransferases (transaminase) AST (GOT), ALT (GPT) 8-Aminolevulinate synthase Protein catabolism Heme synthesis MCC isoniazid therapy Sideroblastic anemia Cheilosis or stomatitis (cracking or scaling of lip borders and corners of the mouth) Convulsions... 

Formation of the Initial Cyclitol. The first steps of FOR production are the same as in the biosynthesis of STR in short, the first step in FOR biosynthesis is postulated to be the formation of a myo-inositol monophosphate (D-myo-inositol-3-phosphate or L-myo-inositol-1-phosphate) via the cellular l-myo-inositol-1-phosphate synthase as in the STR pathway (Ca pathway see Section 2.2.1.2). As in the itr-Att-clusters, no gene for this enzyme has been found in the/or-cluster. As a second step in FOR biosynthesis the dephosphorylation of D-myo-inositol-3-phosphate via an inositolmonophosphate phosphatase has to follow. A putative gene product with this activity is that of the ForA protein (cf. Tables 2.17 and 2.18). The cyclitol is postulated to be first converted via two enzymes, a cluster-encoded myo-inositol 3-dehydrogenase (ForG member of the GFO/IDH/MocA oxidoreductase family) and the L-glutamine icy//o-3-inosose 3-aminotransferase (ketocyclitol aminotransferase I ForS), to icy//o-inosamine (3-deoxy-3-amino- cy/to-mositol). 

K6. Keller, N., Richardson, U. I., and Yates, F. E., Protein binding and the biological activity of corticosteroids in vivo induction of hepatic and pancreatic alanine aminotransferases by corticosteroids in normal and estrogen-treated rats. Endocrinology 84, 49-62 (1969). 

Prephenate aminotransferase The specific activity of this enzyme in Supernatant I was 19.2 nmol/min/mg, and the total activity was retained in Supernatant II following centrifugation at 60,000g. The homogenized pellet (about 300 mg of protein) exhibited enzyme activity (specific activity of... 

Hepatic Effects. Carbon tetrachloride has been known for many years to be a powerful hepatotoxic agent in humans and in animals. The principal clinical signs of liver injury in humans who inhale carbon tetrachloride are a swollen and tender liver, elevated levels of hepatic enzyme (aspartate aminotransferase) in the serum, elevated serum bilirubin levels and the appearance of jaundice, and decreased serum levels of proteins such as albumin and fibrinogen (Ashe and Sailer 1942 McGuire 1932 New et al. 1962 Norwood et al. 1950 Straus 1954). In cases of acute lethal exposures, autopsy generally reveals marked liver necrosis with pronounced steatosis (Jennings 1955 Markham 1967 Smetana 1939), and repeated or chronic exposures leads in some cases to fibrosis or cirrhosis (McDermott and Hardy 1963). 

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