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Descriptor English: Cystathionine beta-Synthase
Descriptor Spanish: Cistationina betasintasa
Descriptor cistationina beta-sintasa
Entry term(s) betationasa
serina sulfhidrasa
Scope note: Enzima multifuncional que requiere fosfato de piridoxal. En la segunda etapa de la biosíntesis de cisteína cataliza la reacción de homocisteína con serina para formar cistationina con eliminación de agua. La deficiencia de esta enzima conduce a HIPERHOMOCISTEINEMIA y HOMOCISTINURIA. EC 4.2.1.22.
Descriptor Portuguese: Cistationina beta-Sintase
Descriptor French: Cystathionine beta-synthase
Entry term(s): Cystathionine Synthetase
Cystathionine beta Synthase
Serine Sulfhydrase
Sulfhydrase, Serine
Synthetase, Cystathionine
beta Thionase
beta-Synthase, Cystathionine
beta-Thionase
Tree number(s): D08.811.520.241.300.200
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D003541
Scope note: 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.
Annotation: /defic: consider also HOMOCYSTINURIA or HYPERHOMOCYSTEINEMIA
Allowable Qualifiers: AD administration & dosage
AE adverse effects
AI antagonists & inhibitors
AN analysis
BI biosynthesis
BL blood
CF cerebrospinal fluid
CH chemistry
CL classification
CS chemical synthesis
DE drug effects
DF deficiency
EC economics
GE genetics
HI history
IM immunology
IP isolation & purification
ME metabolism
PD pharmacology
PH physiology
PK pharmacokinetics
PO poisoning
RE radiation effects
SD supply & distribution
ST standards
TO toxicity
TU therapeutic use
UL ultrastructure
UR urine
Registry Number: EC 4.2.1.22
CAS Type 1 Name: L-Serine hydro-lyase (adding homocysteine)
Previous Indexing: Homocysteine (1973-1974)
Hydro-Lyases (1973-1974)
Serine (1973-1974)
Public MeSH Note: 1991; see HYDRO-LYASES 1975-1990
History Note: 1991(1975)
Related: Homocystinuria MeSH
DeCS ID: 3560
Unique ID: D003541
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1974/11/11
Revision Date: 2003/07/09
Cystathionine beta-Synthase - Preferred
Concept UI M0005533
Scope note 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.
Preferred term Cystathionine beta-Synthase
Entry term(s) Cystathionine Synthetase
Cystathionine beta Synthase
Serine Sulfhydrase
Sulfhydrase, Serine
Synthetase, Cystathionine
beta Thionase
beta-Synthase, Cystathionine
beta-Thionase



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