In vitro modeling of experimental succinic semialdehyde dehydrogenase deficiency (SSADHD) using brain-derived neural stem cells
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DOI: 10.1371/journal.pone.0186919
Abstract: We explored the utility of neural stem cells (NSCs) as an in vitro model for evaluating preclinical therapeutics in succinic semialdehyde dehydrogenase-deficient (SSADHD) mice. NSCs were obtained from aldh5a1+/+ and aldh5a1-/- mice (aldh5a1 = aldehyde… read more here.
Keywords: dehydrogenase; ssadhd; semialdehyde dehydrogenase; succinic semialdehyde ... See more keywords
Understanding the Molecular Mechanisms of Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD): Towards the Development of SSADH-Targeted Medicine
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DOI: 10.3390/ijms23052606
Abstract: Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare genetic disorder caused by inefficient metabolic breakdown of the major inhibitory neurotransmitter, γ-aminobutyric acid (GABA). Pathologic brain accumulation of GABA and γ-hydroxybutyrate (GHB), a neuroactive by-product of… read more here.
Keywords: dehydrogenase deficiency; semialdehyde dehydrogenase; deficiency ssadhd; ssadhd ... See more keywords