Search on: BETA THIONASE 
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Descriptor English:   Cystathionine beta-Synthase 
Descriptor Spanish:   Cistationina betasintasa 
Descriptor Portuguese:   Cistationina beta-Sintase 
Synonyms English:   Cystathionine Synthetase
Cystathionine beta Synthase
Sulfhydrase, Serine
Synthetase, Cystathionine
beta Thionase
beta-Synthase, Cystathionine
beta-Thionase
Serine Sulfhydrase  
Tree Number:   D08.811.520.241.300.200
Definition English:   A multifunctional pyridoxal phosphate enzyme. In the second stage of cysteine biosynthesis it catalyzes the reaction of homocysteine with serine to form cystathionine with the elimination of water. Deficiency of this enzyme leads to HYPERHOMOCYSTEINEMIA and HOMOCYSTINURIA. EC 4.2.1.22. 
Indexing Annotation English:   /defic: consider also HOMOCYSTINURIA or HYPERHOMOCYSTEINEMIA
See Related English:   Homocystinuria
 
History Note English:   1991(1975) 
Allowable Qualifiers English:  
AD administration & dosage AE adverse effects
AN analysis AI antagonists & inhibitors
BI biosynthesis BL blood
CF cerebrospinal fluid CS chemical synthesis
CH chemistry CL classification
CT contraindications DF deficiency
DE drug effects EC economics
GE genetics HI history
IM immunology IP isolation & purification
ME metabolism PK pharmacokinetics
PD pharmacology PH physiology
PO poisoning RE radiation effects
SE secretion ST standards
SD supply & distribution TU therapeutic use
TO toxicity UL ultrastructure
UR urine  
Record Number:   3560 
Unique Identifier:   D003541 

Occurrence in VHL:
 

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