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Computational structural genomics and clinical evidence suggest BCKDK gain-of-function may cause a potentially asymptomatic maple syrup urine disease phenotype. [PDF]

JIMD Rep
Singh E, Chi YI, Kopesky J, Zimmerman M, Urrutia R, Basel D, Schwoerer JS.   +6 more
europepmc   +1 more source

[Maple syrup urine disease].

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke, 1970
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Quantitation of alkylpyrazines in maple syrup, maple flavors and non-maple syrups

Food Research International, 1994
Abstract A modified solvent extraction method was developed and evaluated for the quantitation of pyrazine compounds in maple syrup by capillary gas liquid chromatography. The content of total identified pyrazines in the amber, medium and light maple syrups was 48·89 ng/g, 70·68 ng/g and 57·29 ng/g, respectively.
V Yaylayan
exaly   +2 more sources

Microwave Processing of Maple Sap to Maple Syrup and Maple Syrup Products

Journal of Microwave Power and Electromagnetic Energy, 2001
A study of the physical process of concentration of maple sap to maple syrup and preparation of maple syrup products by microwave heating is described. Duty cycles of 60, 75 and 100% were used for the microwave application. During the process, some of the drying kinetics are discussed, including the reduction of moisture content with time, the progress
G S V Raghavan
exaly   +3 more sources

MAPLE SYRUP

, 2012
Dr. Lisa Mitchell, Associate Professor of South Asia Studies, has guided her students to create voiceover videos as class assignments for a large undergraduate class (ANTH 063 / SAST 063 / HIST 087) in South Asia Studies.
Sekar, Aaditya, Amoo, Benedict, Swaszek, Luke   +2 more
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Detection of Inulin, a Prebiotic Polysaccharide, in Maple Syrup

Journal of Agricultural and Food Chemistry, 2016
Maple syrup is a widely consumed plant-derived natural sweetener produced by concentrating xylem sap collected from certain maple (Acer) species. During thermal evaporation of water, natural phytochemical components are concentrated in maple syrup.
Jiadong Sun, Hang Ma, Navindra P Seeram   +2 more
exaly   +3 more sources

The treatment of maple syrup urine disease

The Journal of Pediatrics, 1969
Summary The early management of 3 infants with maple syrup urine disease is described with particular reference to variations in daily requirements for branched-chain amino acids. The use of a new dry base mix consisting of a dextrimaltose-corn oil-mineral mix, an iron-vitamin mix, and an amino acid mix as a formula base has facilitated earlier home ...
S I, Goodman, S, Pollak, B, Miles, D, O'Brien   +3 more
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Hypoglycemia in Maple Syrup Urine Disease

Archives of Pediatrics & Adolescent Medicine, 1967
HYPOGLYCEMIA associated with maple syrup urine disease has been observed by MacKenzie and Woolf, 1 Silberman, et al, 2 Lonsdale and Barber, 3 and Menkes (oral communication, 1966). Several investigators have speculated as to whether the mechanism resembles that of leucine sensitive hypoglycemia as originally described by Cochrane.
G N, Donnell, E, Lieberman, K N, Shaw, R, Koch   +3 more
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Maple syrup disease

Neurology, 1959
A mixture of alpha-keto acids, the most important of which has been identified as alpha-ketoisocaproic acid, was found to be excreted in large quantities in the urine of beta-methyl-n-valeric acid were present in smaller amounts. Their excretion would suggest an impairment of the degradative metabolism of the branched-chain amino acids ...
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