Maple Syrup Urine Disease

Overview

Journal Arch Dis Child

Specialty Pediatrics

Date 1961 Jun 1

PMID 13733337

Citations 5

Authors

A D PATRICK

Affiliations

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Citing Articles

[MAPLE SYRUP DISEASE WITH FAMILIAL INCIDENCE].

DIEZEL P, Martin K Virchows Arch Pathol Anat Physiol Klin Med. 1964; 337:425-45.

PMID: 14217031

Studies in maple syrup urine disease.

DENT C, WESTALL R Arch Dis Child. 1961; 36:259-68.

PMID: 13721928 PMC: 2012780. DOI: 10.1136/adc.36.187.259.

Synthesis of essential amino acids from their alpha-keto analogues by perfused rat liver and muscle.

Walser M, Lund P, Ruderman N, Coulter A J Clin Invest. 1973; 52(11):2865-77.

PMID: 4748513 PMC: 302555. DOI: 10.1172/JCI107483.

Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain.

Patel M Biochem J. 1974; 144(1):91-7.

PMID: 4462577 PMC: 1168468. DOI: 10.1042/bj1440091.

Differential effects of 2-oxo acids on pyruvate utilization and fatty acid synthesis in rat brain.

Clark J, Land J Biochem J. 1974; 140(1):25-9.

PMID: 4155948 PMC: 1167967. DOI: 10.1042/bj1400025.

References

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EL HAWARY M, THOMPSON R . Separation and estimation of blood keto acids by paper chromatography. Biochem J. 1953; 53(3):340-7. PMC: 1198155. DOI: 10.1042/bj0530340. View

MENKES J, HURST P, Craig J . A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance. Pediatrics. 1954; 14(5):462-7. View

Smith A, Strang L . An inborn error of metabolism with the urinary excretion of alpha-hydroxy-butyric acid and phenylpyruvic acid. Arch Dis Child. 1958; 33(168):109-13. PMC: 2012215. DOI: 10.1136/adc.33.168.109. View

MACKENZIE D, WOOLF L . Maple syrup urine disease; an inborn error of the metabolism of valine, leucine, and isoleucine associated with gross mental deficiency. Br Med J. 1959; 1(5114):90-1. PMC: 1992202. DOI: 10.1136/bmj.1.5114.90. View