Inhibition of brain energy metabolism by the α-keto acids accumulating in maple syrup urine disease

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1639, Issue 3, 2003, Pages 232-238

  Sgaravatti, Angela M ^b   Rosa, Rafael B ^b   Schuck, Patrícia F ^b   Ribeiro, César A J ^b   Wannmacher, Clóvis M D ^b   Wyse, Angela T S ^b   Dutra Filho, Carlos S ^b   Wajner, Moacir ^a  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b NONE

Author keywords

Energy metabolism; Maple syrup urine disease; Keto methylvaleric acid; Ketoisocaproic acid; Ketoisovaleric acid

Indexed keywords

2 OXOACID; 2 OXOISOCAPROIC ACID; 2 OXOISOVALERIC ACID; 3 METHYL 2 OXOVALERIC ACID; ACETIC ACID; BICARBONATE; BUFFER; CARBON DIOXIDE; GLUCOSE; LACTIC ACID; RINGER SOLUTION; SUCCINATE DEHYDROGENASE; UNCLASSIFIED DRUG; VALERIC ACID DERIVATIVE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BRAIN CORTEX; BRAIN DYSFUNCTION; CONTROLLED STUDY; DRUG EFFECT; ENERGY METABOLISM; GLUCOSE BRAIN LEVEL; GLUCOSE TRANSPORT; GLUCOSE UTILIZATION; IN VITRO STUDY; INCUBATION TIME; INHIBITION KINETICS; MAPLE SYRUP URINE DISEASE; METABOLIC DISORDER; METABOLITE; NONHUMAN; PARAMETER; PATHOPHYSIOLOGY; PRIORITY JOURNAL; PRISM; RAT; RAT STRAIN; RESPIRATORY CHAIN; STATISTICAL SIGNIFICANCE;

ACER;

EID: 0242543265     PISSN: 09254439     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbadis.2003.09.010     Document Type: Article

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