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donates an “activated” methyl group for syntheses provides sulfur for synthesis of cysteine provides the activated amine for polyamine synthesis |
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constituent of glutathione (-Glu-Cys-Gly) precursor of taurine source of sulfate (SO42- ) for PAPS (activated sulfate |
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3’-phosphoadenosine 5’phosphosulfate Synthesis of iron-sulfur proteins Sulfation of oligosaccharides such as heparin sulfate Sulfation of hormones, including steroids, T3, T4 |
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| Methionine adenosyltransferase |
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Definition
| catalyzes SAM production from Methionine, needs ATP, releases PPi and Pi |
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| formed when a methyl group is donated to SAM |
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| released from S-adenosylhomocysteine and can be remethylated to methionine, an analog of cystein with an extra CH2 in its carbon backbone |
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| methylated product of guanidinoacetate |
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| the methylated product of phosphatidylethanolamine |
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| methylated product of acetylserotonin |
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| sarcosine (N methylglycine) |
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Definition
| methylated product of glycine |
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Definition
| synthesis of this requires three successive methylation reactions |
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| provides immediate energy in muscle, ATP can then resynthesize |
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| 24 hour urine concentration is a function of muscle mass, elevated plasma levels indicates impaired renal function |
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| arginine, glycine, and SAM |
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Definition
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| GAMT, AGAT, and SLC6A8 deficiency |
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Definition
| the 3 cerebral creatine deficiency syndromes |
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Definition
Mental retardation and seizures Approximately 80% of individuals have a behavior disorder that can include autistic behaviors and self-mutilation Onset is between ages three months and three years. |
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| oxidized dimer of homocysteine |
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Definition
| used to designate a mixture of homocysteine and homocystine |
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| methylation of homocysteine to form methionine requires |
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| can also donate a ch3 to B12, liver and kidney, oxidation product of choline |
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| intermediate in cystein synthesis pathway, fromed by condensation of homocysteine and serine, cleaved by hydrolysis |
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| tripeptide synthesized by sequential addition of cysteine and then glycine to glutamate, protects from oxidation, detoxify h2o2, FA peroxides, and xenobiotis, synthesis of leukotriene C4 and contributs to amino acid absorption |
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| a selenium containing enzyme which removes h2o2 and minimized oxidative damage |
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| reduced GSH is regenerated from oxidized GSSG by |
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| a peptidyl leudotriene which contains glutathione, formed via the lipoxygenase pathway from arachidonic acid, potent stimulant of bronchoconstriction |
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| cycle that occurs in kidney and intestine, transpeptidase attaches extracellular AA to GSH to facilitate AA transfer into the cell |
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Definition
| an amino sulfonic acid, sulfate instead of carboxyl, not used for protein synthesis |
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| oxidation, decarboxylation, oxidation |
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Definition
| reactions to produce taurine from cysteine |
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Definition
| although adults form both glycine and taurine conjugates for bile salts, neonates only use |
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abundant intracellularly in retina and leukocytes, brain and retinal development, osmoregulation, modulation of Ca processes, and membrane stabilization May regulate the expression of genes required for glucose-stimulated insulin secretion May enhance peripheral insulin sensitivity May decrease atherosclerosis |
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| oxidative stress or iron overload |
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Definition
| taurine may protect against |
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Definition
| have a lower store or taurine, inefficient reabsorption of taurine, low activity of cystathionase |
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