Purpose

      Types of Aminoacidurias

There are two types of aminoacidurias. Primary or overflow aminoaciduria results from deficiencies in the enzymes necessary to metabolize amino acids. Overflow aminoaciduria is best detected by a blood plasma test.

Secondary or renal aminoaciduria occurs because of a congenital defect in the amino acid transport system in the tubules of the kidneys. This produces increased amino acids in the urine. Blood and urine test in combination are used to determine if the aminoaciduria is of the overflow or renal type. Urine tests are also used to monitor specific amino acid disorders.

Newborns are screened for amino acid disorders. Young children with acidosis (accumulation of acid in the body), severe vomiting and diarrhea, or urine with an abnormal color or odor, are also screened with a urine test for specific amino acid levels.

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Structure of Amino Acids
Amino Acid Properties

Classes of Amino Acids
Amino Acid Disorders Screening
Functions and Non-functions in proteins