Succinyladenosine
public_id
FDB022314
IUPAC 名称
(2S)-2-({9-[(2R,3R,4S,5R)-3,4-dihydroxy-5-(hydroxymethyl)oxolan-2-yl]-9H-purin-6-yl}amino)butanedioic acid
描述
Succinyladenosine (SAdo) is one of the dephosphorylated enzyme substrate that accumulates in body fluids of patients with adenylosuccinate lyase (ADSL) deficiency, the other being 5-amino-4-imidazole-N-succinocarboxamide riboside (SAICAr). ADSL is an inherited metabolic disease characterized by various degrees of psychomotor retardation. (PMID 15902552) The severity of the clinical presentation correlates with a low S-Ado/SAICAr ratio in body fluids. (PMID: 15571235) Normally Succinyladenosine is not found in blood or CSF but may be detected in trace amounts in urine. (OMIM 103050) [HMDB]
CAS号
4542-23-8
异构
OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C(N[C@@H](CC(O)=O)C(O)=O)N=CN=C12
InChI标识符
InChI=1S/C14H17N5O8/c20-2-6-9(23)10(24)13(27-6)19-4-17-8-11(15-3-16-12(8)19)18-5(14(25)26)1-7(21)22/h3-6,9-10,13,20,23-24H,1-2H2,(H,21,22)(H,25,26)(H,15,16,18)/t5-,6+,9+,10+,13+/m0/s1
VKGZCEJTCKHMRL-VWJPMABRSA-N
平均分子量
383.107712545
id
22477
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