Monitoring ?-hydroxybutyric acid levels in succinate-semialdehyde dehydrogenase deficiency

2004 ◽  
Vol 55 (4) ◽  
pp. 599-599 ◽  
Author(s):  
Phillip L. Pearl ◽  
Andrea Gropman
Keyword(s):  
Dehydrogenase Deficiency ◽  
Hydroxybutyric Acid ◽  
Semialdehyde Dehydrogenase ◽  
Succinate Semialdehyde

Succinate semialdehyde dehydrogenase deficiency does not down-regulate γ-hydroxybutyric acid binding sites in the mouse brain

2006 ◽  
Vol 88 (1) ◽  
pp. 86-89 ◽  
Author(s):  
Ashok K. Mehta ◽  
Georgianna G. Gould ◽  
Maneesh Gupta ◽  
Lawrence P. Carter ◽  
K. Michael Gibson ◽  
...  
Keyword(s):  
Mouse Brain ◽  
Binding Sites ◽  
Dehydrogenase Deficiency ◽  
Hydroxybutyric Acid ◽  
Semialdehyde Dehydrogenase ◽  
Succinate Semialdehyde

Expression profiling reveals multiple myelin alterations in murine succinate semialdehyde dehydrogenase deficiency

2006 ◽  
Vol 29 (1) ◽  
pp. 143-156 ◽  
Author(s):  
Elizabeth A. Donarum ◽  
Dietrich A. Stephan ◽  
Kay Larkin ◽  
Eric J. Murphy ◽  
Maneesh Gupta ◽  
...  
Keyword(s):  
Expression Profiling ◽  
Dehydrogenase Deficiency ◽  
Semialdehyde Dehydrogenase ◽  
Succinate Semialdehyde

Prenatal diagnosis of succinate semialdehyde dehydrogenase deficiency in non-identical twins

2002 ◽  
Vol 25 (6) ◽  
pp. 517-518 ◽  
Author(s):  
I. A. Aligianis ◽  
P. A. Farndon ◽  
R. G. F. Gray ◽  
S. K. Heath ◽  
M. Kilby ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Dehydrogenase Deficiency ◽  
Identical Twins ◽  
Semialdehyde Dehydrogenase ◽  
Succinate Semialdehyde

scholarly journals New Insights into γ-Aminobutyric Acid Catabolism: Evidence for γ-Hydroxybutyric Acid and Polyhydroxybutyrate Synthesis in Saccharomyces cerevisiae

2009 ◽  
Vol 75 (13) ◽  
pp. 4231-4239 ◽  
Author(s):  
Benoît Bach ◽  
Emmanuelle Meudec ◽  
Jean-Paul Lepoutre ◽  
Tristan Rossignol ◽  
Bruno Blondin ◽  
...  
Keyword(s):  
Redox Homeostasis ◽  
Industrial Applications ◽  
Reaction Yield ◽  
Nitrogen Storage ◽  
Alternative Route ◽  
Hydroxybutyric Acid ◽  
Anaerobic Conditions ◽  
Tolerance Mechanisms ◽  
Semialdehyde Dehydrogenase ◽  
Succinate Semialdehyde

ABSTRACT The γ-aminobutyrate (GABA) shunt, an alternative route for the conversion of α-ketoglutarate to succinate, involves the glutamate decarboxylase Gad1p, the GABA transaminase Uga1p and the succinate semialdehyde dehydrogenase Uga2p. This pathway has been extensively described in plants and animals, but its function in yeast remains unclear. We show that the flux through Gad1p is insignificant during fermentation in rich sugar-containing medium, excluding a role for this pathway in redox homeostasis under anaerobic conditions or sugar stress. However, we found that up to 4 g of exogenous GABA/liter was efficiently consumed by yeast. We studied the fate of this consumed GABA. Most was converted into succinate, with a reaction yield of 0.7 mol/mol. We also showed that a large proportion of GABA was stored within cells, indicating a possible role for this molecule in stress tolerance mechanisms or nitrogen storage. Furthermore, based on enzymatic and metabolic evidence, we identified an alternative route for GABA catabolism, involving the reduction of succinate-semialdehyde into γ-hydroxybutyric acid and the polymerization of γ-hydroxybutyric acid to form poly-(3-hydroxybutyric acid-co-4-hydroxybutyric acid). This study provides the first demonstration of a native route for the formation of this polymer in yeast. Our findings shed new light on the GABA pathway and open up new opportunities for industrial applications.

Murine succinate semialdehyde dehydrogenase deficiency

2003 ◽  
Vol 54 (S6) ◽  
pp. S81-S90 ◽  
Author(s):  
Maneesh Gupta ◽  
Boris M. Hogema ◽  
Markus Grompe ◽  
Teodoro G. Bottiglieri ◽  
Alessandra Concas ◽  
...  
Keyword(s):  
Dehydrogenase Deficiency ◽  
Semialdehyde Dehydrogenase ◽  
Succinate Semialdehyde
Sign in / Sign up

Export Citation Format

Share Document