Literature DB >> 4813956

Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.

G Lancaster, O A Mamer, C R Scriver.   

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Year:  1974        PMID： 4813956     DOI： 10.1016/0026-0495(74)90064-x

Source DB:  PubMed          Journal:  Metabolism        ISSN： 0026-0495            Impact factor:   8.694


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  10 in total

1.  Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination.

Authors:  E Treacy; C L Clow; T R Reade; D Chitayat; O A Mamer; C R Scriver
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

2.  Management of maple syrup urine disease in Canada. Committee for improvement of Hereditary Disease Management.

Authors: 
Journal:  Can Med Assoc J       Date:  1976-11-20       Impact factor: 8.262

3.  Effects of 4-methyl-2-methylenevalerate, a non-metabolizable analogue of 2-ketoisocaproate, on insulin secretion and metabolism in ob/ob mouse pancreatic islets.

Authors:  S Lenzen; U Panten
Journal:  Experientia       Date:  1982-09-15

4.  Peritoneal dialysis in maple-syrup-urine disease: studies on branched-chain amino and keto acids.

Authors:  U Wendel; K Becker; H Przyrembel; M Bulla; C Manegold; A Mench-Hoinowski; U Langenbeck
Journal:  Eur J Pediatr       Date:  1980-06       Impact factor: 3.183

5.  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

6.  Artefactual pyruvate and 2-oxobutyrate produced by trimethylsilylation of methylmalonic and ethylmalonic acids in the presence of oxygen.

Authors:  O A Mamer; L Choiniere; D Boismenu; F Lepine
Journal:  J Inherit Metab Dis       Date:  1999-10       Impact factor: 4.982

7.  Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease.

Authors:  Y Shigematsu; K Kikuchi; T Momoi; M Sudo; Y Kikawa; K Nosaka; M Kuriyama; S Haruki; K Sanada; N Hamano
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

8.  Properties of the testicular lactate dehydrogenase isoenzyme.

Authors:  A Blanco; C Burgos; N M Gerez de Burgos; E E Montamat
Journal:  Biochem J       Date:  1976-02-01       Impact factor: 3.857

9.  Effects of alfa-hydroxy-isocaproic acid on body composition, DOMS and performance in athletes.

Authors:  Antti A Mero; Tuomo Ojala; Juha J Hulmi; Risto Puurtinen; Tuomo Am Karila; Timo Seppälä
Journal:  J Int Soc Sports Nutr       Date:  2010-01-05       Impact factor: 5.150

10.  Identification of 2-hydroxyisocaproic acid production in lactic acid bacteria and evaluation of microbial dynamics during kimchi ripening.

Authors:  Boyeon Park; Hyelyeon Hwang; Ji Yoon Chang; Sung Wook Hong; Se Hee Lee; Min Young Jung; Sung-Oh Sohn; Hae Woong Park; Jong-Hee Lee
Journal:  Sci Rep       Date:  2017-09-07       Impact factor: 4.379
  10 in total

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