Transport of Amino Acids Into Cells

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Chapter: Biochemistry : Amino Acids: Disposal of Nitrogen

The concentration of free amino acids in the extracellular fluids is significantly lower than that within the cells of the body.


TRANSPORT OF AMINO ACIDS INTO CELLS

The concentration of free amino acids in the extracellular fluids is significantly lower than that within the cells of the body. This concentration gradient is maintained because active transport systems, driven by the hydrolysis of ATP, are required for movement of amino acids from the extracellular space into cells. At least seven different transport systems are known that have overlapping specificities for different amino acids. Because the small intestine and the proximal tubule of the kidney have common transport systems for amino acid uptake, a defect in any one of these systems results in an inability to absorb particular amino acids into the gut and into the kidney tubules. For example, one system is responsible for the uptake of cystine and the dibasic amino acids, ornithine, arginine, and lysine (represented as “COAL”). In the inherited disorder cystinuria, this carrier system is defective, and all four amino acids appear in the urine (Figure 19.6). Cystinuria occurs at a frequency of 1 in 7,000 individuals, making it one of the most common inherited diseases and the most common genetic error of amino acid transport. The disease expresses itself clinically by the precipitation of cystine to form kidney stones (calculi), which can block the urinary tract. Oral hydration is an important part of treatment for this disorder. [Note: Defects in the transport of tryptophan can result in Hartnup disorder and pellagra-like dermatologic and neurologic symptoms.]


Figure 19.6 Genetic defect seen in cystinuria. [Note: Cystinuria is distinct from cystinosis, a rare defect in the transport of cystine out of lysosomes that results in the formation of cystine crystals within the lysosome and tissue damage.]

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