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Intracellular metabolism of cystine, as it happens with all amino acids, requires its transport across the cell membrane. After degradation of endocytosed protein to cystine within lysosomes, it is normally transported to the cytosol. But if there is a defect in the carrier protein, cystine is accumulated in lysosomes.
The recommended daily intakes for children aged three years and older is 10% to 20% higher than adult levels and those for infants can be as much as 150% higher in the first year of life. Cysteine (or sulfur-containing amino acids), tyrosine (or aromatic amino acids), and arginine are always required by infants and growing children.
The presence of cystine in urine is often indicative of amino acid reabsorption defects. Cystinuria has been reported to occur in dogs. [14] In humans the excretion of high levels of cystine crystals can be indicative of cystinosis, a rare genetic disease. Cystine stones account for about 1-2% of kidney stone disease in adults. [15] [16]
Cystinuria is an inherited autosomal recessive disease [1] characterized by high concentrations of the amino acid cystine in the urine, leading to the formation of cystine stones in the kidneys, ureters, and bladder. It is a type of aminoaciduria. "Cystine", not "cysteine," is implicated in this disease; the former is a dimer of the latter.
For adults, the recommended minimum amounts of each essential amino acid varies from 4 to 39 milligrams per kilogram of body weight per day. To be of good quality, protein only needs to come from a wide variety of foods; there is neither a need to mix animal and plant food together nor a need to complement specific plant foods, such as rice and ...
These high-fiber foods are delicious and expert-approved. Incorporate these picks into your diet for a hefty dose the many benefits fiber promises. 20 expert-approved high-fiber foods to keep you ...
The recommended daily allowance (RDA) of methionine (combined with cysteine) for adults is set at 13–14 mg kg-1 day-1 (13–14 mg per kg of body weight per day), but some researchers have argued that this figure is too low, and should more appropriately be 25 mg kg-1 day-1. [1]
Cystathionase catalyzes cystathionine to cysteine and α-ketobutyrate. [3] Cysteine is an essential amino acid and its conversion from cystathionine occurs in the trans-sulfuration pathway. The availability of cysteine is necessary for the synthesis of an important anti-oxidant, glutathione. [ 2 ]