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The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease.
Pediatr Res. 1984 Sep; 18(9):851-3.PR

Abstract

Plasma branched chain amino acid levels and their respective ketoacid analogues were determined in seven maple syrup urine disease patients ranging in age from 12 h to 12 years. One hundred one pairs were analyzed. There was a high degree of correlation between the amino acid and its ketoacid analogue at every amino acid level. The coefficient of correlation (0.84) was highest for leucine-alpha-ketoisocaproic acid. The ratio of ketoacid analogue to amino acid, (0.87), was also the greatest for leucine. The close correlation implies that adequate monitoring for therapy can be accomplished by the use of the technically simpler and more rapid determination of the plasma branched chain amino acids.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

6483508

Citation

Snyderman, S E., et al. "The Relationship Between the Branched Chain Amino Acids and Their Alpha-ketoacids in Maple Syrup Urine Disease." Pediatric Research, vol. 18, no. 9, 1984, pp. 851-3.
Snyderman SE, Goldstein F, Sansaricq C, et al. The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease. Pediatr Res. 1984;18(9):851-3.
Snyderman, S. E., Goldstein, F., Sansaricq, C., & Norton, P. M. (1984). The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease. Pediatric Research, 18(9), 851-3.
Snyderman SE, et al. The Relationship Between the Branched Chain Amino Acids and Their Alpha-ketoacids in Maple Syrup Urine Disease. Pediatr Res. 1984;18(9):851-3. PubMed PMID: 6483508.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease. AU - Snyderman,S E, AU - Goldstein,F, AU - Sansaricq,C, AU - Norton,P M, PY - 1984/9/1/pubmed PY - 1984/9/1/medline PY - 1984/9/1/entrez SP - 851 EP - 3 JF - Pediatric research JO - Pediatr Res VL - 18 IS - 9 N2 - Plasma branched chain amino acid levels and their respective ketoacid analogues were determined in seven maple syrup urine disease patients ranging in age from 12 h to 12 years. One hundred one pairs were analyzed. There was a high degree of correlation between the amino acid and its ketoacid analogue at every amino acid level. The coefficient of correlation (0.84) was highest for leucine-alpha-ketoisocaproic acid. The ratio of ketoacid analogue to amino acid, (0.87), was also the greatest for leucine. The close correlation implies that adequate monitoring for therapy can be accomplished by the use of the technically simpler and more rapid determination of the plasma branched chain amino acids. SN - 0031-3998 UR - https://www.unboundmedicine.com/medline/citation/6483508/The_relationship_between_the_branched_chain_amino_acids_and_their_alpha_ketoacids_in_maple_syrup_urine_disease_ L2 - https://doi.org/10.1203/00006450-198409000-00009 DB - PRIME DP - Unbound Medicine ER -