HISTOCHEMICAL LOCALIZATION OF BRAIN SUCCINIC SEMIALDEHYDE DEHYDROGENASE-A γ-AMINOBUTYRIC ACID DEGRADATIVE ENZYME
نویسندگان
چکیده
منابع مشابه
Succinic Semialdehyde Dehydrogenase
The enzyme succinic semialdehyde dehydrogenase from pig brain has been 2000-fold purified by a combination of DEAE-cellulose, hydroxyapatite, and AMPSepharose chromatography. This preparation has a molecular weight of 160,000 and a specific activity of 5.3 pmol/min*mg at 25°C. The inhibition of succinic semialdehyde dehydrogenase by carbonyl compounds, i.e. P-pyridoxal and o-phthalaldehyde was ...
متن کاملCharacterization of succinic semialdehyde dehydrogenase from Aspergillus niger.
The catabolism of fungal 4-aminobutyrate (GABA) occurs via succinic semialdehyde (SSA). Succinic semialdehyde dehydrogenase (SSADH) from the acidogenic fungus Aspergillus niger was purified from GABA grown mycelia to the highest specific activity of 277 nmol min(-1) mg(-1), using phenyl Sepharose and DEAE Sephacel chromatography. The purified enzyme was specific for its substrates SSA and NAD+....
متن کاملTargeted screening of succinic semialdehyde dehydrogenase deficiency (SSADHD) employing an enzymatic assay for γ-hydroxybutyric acid (GHB) in biofluids
HYPOTHESIS An enzymatic assay for quantification of γ-hydroxybutyric acid (GHB) in biofluids can be employed for targeted screening of succinic semialdehyde dehydrogenase deficiency (SSADHD) in selected populations. RATIONALE We used a two-tiered study approach, in which the first study (proof of concept) examined 7 urine samples derived from patients with SSADHD and 5 controls, and the secon...
متن کاملHead bobbing due to succinic semialdehyde dehydrogenase deficiency.
A 9-month-old boy had a short history of “yes-yes” head bobbing (video on the Neurology Web site at www. neurology.org). Mild global delay prompted investigation. Imaging (figure, A–C), biochemical, and molecular findings (table e-1) were consistent with succinic semialdehyde dehydrogenase (SSADH) deficiency. SSADH deficiency, a -aminobutyric acid degradation disorder, may be associated with mo...
متن کاملProton MR spectroscopy in succinic semialdehyde dehydrogenase deficiency.
Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare hereditary disorder of the CNS catabolism of gamma-aminobutyric acid (GABA), leading to accumulation of the metabolite 4-hydroxybutyrate (GHB). Here the authors report on 1.5 and 3.0 T proton MR spectroscopy in a patient with SSADH deficiency. A characteristic pattern with clearly elevated GABA levels and traces of GHB was found i...
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ژورنال
عنوان ژورنال: Journal of Histochemistry & Cytochemistry
سال: 1971
ISSN: 0022-1554,1551-5044
DOI: 10.1177/19.7.405