Life Science Encyclopedia

Valinemia is a rare congenital disorder in which urinary and serum valine are elevated without elevation of leucine and isoleucine. It is caused by a deficiency of the enzyme valine transaminase. Symptoms include lack of appetite, vomiting and failure to thrive.

References

• Dancis J, Hutzler J, Tada K, Wada Y, Morikawa T, Arakawa T (1967). "Hypervalinemia. A defect in valine transamination". Pediatrics 39 (6): 813–7. PMID 6067402.
• Tada K, Wada Y, Arakawa T (1967). "Hypervalinemia. Its metabolic lesion and therapeutic approach". Am. J. Dis. Child. 113 (1): 64–7. PMID 6066688.
• Wada Y, Tada K, Minagawa A, Yoshida T, Morikawa T, Okamura T (1963). "Idiopathic hypervalinemia: probably a new entity of inborn error of valine metabolism". Tohoku J. Exp. Med. 81: 46–55. PMID 14077060.