Search on: BETA-THIONASE 
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Descriptor English:   Cystathionine beta-Synthase 
Descriptor Spanish:   Cistationina betasintasa 
Descriptor Portuguese:   Cistationina beta-Sintase 
Synonyms English:   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:  
administration & dosage adverse effects
antagonists & inhibitors analysis
biosynthesis blood
cerebrospinal fluid chemistry
classification chemical synthesis
contraindications drug effects
deficiency diagnostic use
economics genetics
history immunology
isolation & purification metabolism
pharmacology physiology
pharmacokinetics poisoning
radiation effects supply & distribution
secretion standards
toxicity therapeutic use
ultrastructure urine
Record Number:   3560 
Unique Identifier:   D003541 

Occurrence in VHL: 		 

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