Disturbance of Cultured Rat Neuronal Network Activity Depends on Concentration and Ratio of Leucine and α-Ketoisocaproate: Implication for Acute Encephalopathy of Maple Syrup Urine Disease

Görtz, Philipp; Köller, Hubertus; Schwahn, Bernd; Wendel, U.; Siebler, Mario

doi:10.1203/01.pdr.0000047521.50656.16

Cited by 11 publications

(1 citation statement)

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“…The deleterious metabolic consequences of unbalanced ketoacid pools in general are common to all organisms (4,10,20,23,27,30,32,61). We envision YjgF having a broad affinity range and suggest that while YjgF prevents the toxicity of product X, other ␣-ketoacids could be similarly sequestered.…”

Section: Discussionmentioning

confidence: 99%

Reduced Transaminase B (IlvE) Activity Caused by the Lack ofyjgFIs Dependent on the Status of Threonine Deaminase (IlvA) inSalmonella entericaSerovar Typhimurium

2004

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The YjgF/YER057c/UK114 family is a highly conserved class of proteins that is represented in the three domains of life. Thus far, a biochemical function demonstrated for these proteins in vivo or in vitro has yet to be defined. In several organisms, strains lacking a YjgF homolog have a defect in branched-chain amino acid biosynthesis. This study probes the connection between yjgF and isoleucine biosynthesis in Salmonella enterica. In strains lacking yjgF the specific activity of transaminase B, catalyzing the last step in the synthesis of isoleucine, was reduced. In the absence of yjgF, transaminase B activity could be restored by inhibiting threonine deaminase, the first enzymatic step in isoleucine biosynthesis. Strains lacking yjgF showed an increased sensitivity to sulfometruron methyl, a potent inhibitor of acetolactate synthase. Based on work described here and structural reports in the literature, we suggest a working model in which YjgF has a role in protecting the cell from toxic effects of imbalanced ketoacid pools.

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