Literature DB >> 3677437

New conjugated urinary metabolites in intermediate type maple syrup urine disease.

L Hagenfeldt1, A S Naglo.   

Abstract

N-Lactylvaline, N-lactylleucine, N-lactylisoleucine and the N-2-hydroxyisovaleryl conjugates of glycine, valine, leucine and isoleucine have been identified in urine from a patient with the intermediate type of maple syrup urine disease.

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Year:  1987        PMID： 3677437     DOI： 10.1016/0009-8981(87)90395-0

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  4 in total

Review 1.  Biosynthesis, degradation and pharmacological importance of the fatty acid amides.

Authors:  Emma K Farrell; David J Merkler
Journal:  Drug Discov Today       Date:  2008-04-03       Impact factor: 7.851

2.  N-lactoyl-amino acids are ubiquitous metabolites that originate from CNDP2-mediated reverse proteolysis of lactate and amino acids.

Authors:  Robert S Jansen; Ruben Addie; Remco Merkx; Alexander Fish; Sunny Mahakena; Onno B Bleijerveld; Maarten Altelaar; Lodewijk IJlst; Ronald J Wanders; P Borst; Koen van de Wetering
Journal:  Proc Natl Acad Sci U S A       Date:  2015-05-11       Impact factor: 11.205

3.  Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.

Authors:  P Schadewaldt; H W Hammen; A C Ott; U Wendel
Journal:  J Inherit Metab Dis       Date:  1999-08       Impact factor: 4.982

4.  Circulating markers of NADH-reductive stress correlate with mitochondrial disease severity.

Authors:  Rohit Sharma; Bryn Reinstadler; Kristin Engelstad; Owen S Skinner; Erin Stackowitz; Ronald G Haller; Clary B Clish; Kerry Pierce; Melissa A Walker; Robert Fryer; Devin Oglesbee; Xiangling Mao; Dikoma C Shungu; Ashok Khatri; Michio Hirano; Darryl C De Vivo; Vamsi K Mootha
Journal:  J Clin Invest       Date:  2021-01-19       Impact factor: 14.808
  4 in total

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