Solerol (BioDeep_00000027779)

描述信息

Solerol, also known as 4,5-dihydroxyhexanoic acid lactone, is a volatile component originally identified in extracts of grape wines and numerous fruits, with aroma being among the most important factors in determining wine character and quality (PMID: 11052736 ). Solerol (4,5-dihydroxyhexanoic acid lactone) also appears to be a marker for Succinic semialdehyde dehydrogenase (SSADH) deficiency and probably arises from the reaction of succinic semialdehyde with an intermediate in the pyruvate dehydrogenase pathway (PMID: 3126356 ). SSADH is a disorder that arises from a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB), 2,4-dihydroxybutyric acid, adipic acid as well as elevated levels of 4,5-dihydroxyhexanoic acid, and 4,5-dihydroxyhexanoic acid lactone and causing neurological and cognitive disorders of varying severity (PMID: 3126356 ). Children with SSADH deficiency usually manifest with developmental delay, behavioral symptoms, language dysfunction, seizures, hypotonia, extrapyramidal symptoms, and ataxia (PMID: 32055132).
Solerol is a volatile component identified in extracts of grape wines and numerous fruits, with aroma being among the most important factors in determining wine character and quality. (PMID: 11052736). Solerol (4,5-dihydroxyhexanoic acid lactone) also appears to be a marker for Succinic semialdehyde dehydrogenase (SSADH) deficiency and probably arises from the reaction of succinic semialdehyde with an intermediate in the pyruvate dehydrogenase pathway (PMID: 3126356). SSADH is a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB) and causing neurological and cognitive disorders of varying severity. [HMDB]

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