Succinate-semialdehyde dehydrogenase is an enzyme that in humans is encoded by the ALDH5A1 gene. This protein belongs to the aldehyde dehydrogenase family of proteins. This gene encodes a mitochondrial NAD+-dependent succinic semialdehyde dehydrogenase. A deficiency of this enzyme, known as 4-hydroxybutyricaciduria, is a rare inborn error in the metabolism of the neurotransmitter γ-aminobutyric acid (GABA). In response to the defect, physiologic fluids from patients accumulate GHB, a compound with numerous neuromodulatory properties. Two transcript variants encoding distinct isoforms have been identified for this gene.
Western
Western blot analysis of ALDH5A1 in lysates of Human liver and HepG2 using ALDH5A1 antibody.
Immunohistochemistry
Immunohistochemistry analysis of paraffin-embedded Human liver using ALDH5A1 antibody.High pressure and temperature Tris/EDTA (PH 9.0) used for antigen retrieval.The brown color represents the positive signal observed with ALDH5A1 antibody.