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Information on EC 4.2.1.22 - cystathionine beta-synthase and Organism(s) Mus musculus and UniProt Accession Q91WT9
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4.2.1.22 cystathionine beta-synthaseIUBMB Comments
A pyridoxal-phosphate protein. A multifunctional enzyme: catalyses beta-replacement reactions between L-serine, L-cysteine, cysteine thioethers, or some other beta-substituted alpha-L-amino acids, and a variety of mercaptans.
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- 4.2.1.22
- h2s
- sulfide
- homocystinuria
- hyperhomocysteinemia
- artery
-
spacer
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corticobasal
- candida
- mthfr
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carotid
- folate
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transsulfuration
- conidia
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cajal
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- dextrose
- methylenetetrahydrofolate
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bismuth
- hallucinations
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anamorphic
- palsy
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supranuclear
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charles
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remethylation
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reisolated
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conidiophore
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gamma-lyase
-
biodiversity
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hyaline
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chemoreceptor
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gasotransmitter
- 3-mercaptopyruvate
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frontotemporal
- sulfurtransferase
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thromboembolic
- ascospore
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rinsed
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aminooxyacetic
- apraxia
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snrnps
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symptomless
- hydrosulfide
- marxianus
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visuospatial
- 5,10-methylenetetrahydrofolate
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voxel-based
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appressoria
- naocl
- medicine
- diagnostics
- ascus
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phytopathological
- analysis
The taxonomic range for the selected organisms is: Mus musculus
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
Synonyms
cbs, cystathionine beta-synthase, cystathionine-beta-synthase, cnnm2, cystathionine beta synthase, serine sulfhydrylase, ytcbs, serine sulfhydrase, cdcp2, cbs424, 
more
topSYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
Beta-thionase
-
-
-
-
CBS
Cysteine synthase
-
-
-
-
Hemoprotein H-450
-
-
-
-
Methylcysteine synthase
-
-
-
-
Serine sulfhydrase
-
-
-
-
Serine sulfhydrylase
-
-
-
-
Serine sulphhydrase
-
-
-
-
CBS
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
elimination
-
-
-
-
C-S bond formation
-
-
-
-
PATHWAY SOURCE
PATHWAYS
BRENDA
KEGG
-
-, -, -, -
SYSTEMATIC NAME
IUBMB Comments
L-serine hydro-lyase (adding homocysteine; L-cystathionine-forming)
A pyridoxal-phosphate protein. A multifunctional enzyme: catalyses beta-replacement reactions between L-serine, L-cysteine, cysteine thioethers, or some other beta-substituted alpha-L-amino acids, and a variety of mercaptans.
CAS REGISTRY NUMBER
COMMENTARY
9023-99-8
-
SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
L-serine + L-homocysteine
cystathionine + H2O
the enzyme participates in the process of oocyte maturation
-
-
?
additional information
?
-
-
cystathionine beta-synthase is a key enzyme for homocysteine metabolism, it is associated with the generation and/or differentiation of the radial glia/astrocyte linage cells in the developing central nervous system
-
-
?
additional information
?
-
-
hyperhomocysteinaemia is a metabolic disorder associated with the development of premature atherosclerosis. Cystathionine beta-synthase activity is significantly decreased in mice with a plasma homocysteine value greater than 0.015 mg
-
-
?
additional information
?
-
-
role for CBS as a mediator in interactions between oocyte and granulosa cells
-
-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
L-serine + L-homocysteine
cystathionine + H2O
the enzyme participates in the process of oocyte maturation
-
-
?
additional information
?
-
-
cystathionine beta-synthase is a key enzyme for homocysteine metabolism, it is associated with the generation and/or differentiation of the radial glia/astrocyte linage cells in the developing central nervous system
-
-
?
additional information
?
-
-
hyperhomocysteinaemia is a metabolic disorder associated with the development of premature atherosclerosis. Cystathionine beta-synthase activity is significantly decreased in mice with a plasma homocysteine value greater than 0.015 mg
-
-
?
additional information
?
-
-
role for CBS as a mediator in interactions between oocyte and granulosa cells
-
-
?
COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
carbon monoxide
-
binds to the prosthetic heme, stabilizing 6-coordinated CO-Fe(II)-histidine complex to block the activity
ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
Ethionine
-
i.e. 2-amino-4-(ethylthio)butyric acid, an methionine analogue, which is converted to S-adenosyl-ethionine in vivo and activates CBS, treatment increased liver CBS activity 4.0fold in wild-type mice
S-adenosyl-L-methionine
-
the enzyme is allosterically activated by S-adenosyl-L-methionine under normal conditions but is destabilized under pathological conditions
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
cystathionine beta-synthase is associated with the generation and/or differentiation of the radial glia/astrocyte lineage cells in the developing central nervous system
-
hyperhomocysteinaemia is a metabolic disorder associated with the development of premature atherosclerosis. Cystathionine beta-synthase activity is significantly decreased in mice with a plasma homocysteine value greater than 0.015 mg
-
cystathionine beta-synthase is associated with the generation and/or differentiation of the radial glia/astrocyte lineage cells in the developing central nervous system
-
ubiquitously expressed in the ovary with the strongest expression in follicular cells at all stages. In late antral follicles, cystathionine beta-synthase expression is markedly higher in granulosa cells located close to the antrum and in cumulus cells around the oocyte
additional information
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
physiological function
additional information
-
increased immunoreactivity is evident in liver homogenates from mice treated with adenovirus carrying human CBS compared to mice treated with the Ad-lacZ virus. Overexpression of hCBS reduces homocysteine levels significantly by 5.3fold compared to Ad-lacZ treated mice
malfunction
-
hemizygous (+/-) CBS knockout mice are analysed: Significantly higher plasma total homocysteine concentrations occurr in the CBS (+/-) mice than in wild-type cohorts. Female mice of both genotypes have significantly higher plasma total homocysteine concentrations and lower relative CBS mRNA levels than did male mice. During vitamin B6 deficiency, plasma total homocysteine concentrations are significantly elevated. CBS (+/-) mice have a lower plasma cholesterol concentration, and during a taurine- and cysteine-deficient diet, CBS mRNA levels in CBS (+/-) mice are reduced only 13%
malfunction
-
the effects of endogenous elevation of homocysteine on the retina using the cystathionine beta-synthase mutant mouse is determined. Increased retinal homocysteine alters inner and outer retinal layers in cbs homozygous mice and older cbs heterozygous mice, and it primarily affects the cells of the ganglion cell layer in younger heterozygous mice. Elevated retinal homocysteine alters expression of genes involved in endoplasmic reticular stress, N-methyl-D-aspartate (NMDA) receptor activation, cell cycle, and apoptosis
physiological function
-
CBS activity partially regulates endogenous H2S in mice. It contributes significantly to endogenous H2S production in mice, adenovirus-mediated overexpression of CBS in the liver significantly increases circulating levels of H2S, whereas CBS deficiency results in reduced levels. irculating H2S levels are increased by pharmacological activation of CBS in vivo; i.e. in the presence of the endogenous activator
physiological function
-
Cystathionine beta synthase (CBS) is the main contributor to the production of hydrogen sulfide (H2S) in the brain. The CBS/H2S pathway plays an important role in the protection of learning and memory functions in the brain at the level of the hippocampus
physiological function
deletion mutants of conserved domain protein/cyclin M Mg2+ transporter CNNM2, lacking the CBS domains, are unable to promote Mg2+ efflux. The CBS domains of CNNM2 bind to ATP in a Mg2+-dependent manner
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). CBS_MOUSE
561
0
61544
Swiss-Prot
other Location (Reliability: 1)
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
T568I
substitution of amino acid residue in the CBS domains of CNNM2, abrogates Mg2+ efflux and binding of ATP
additional information
-
CBS activity in liver homogenates is reduced and plasma homocysteine levels are elevated in the CBS heterozygous knockout , CBS-/+ animals compared to wild-type littermate control mice. H2S is also significantly reduced by 30% and 46% compared to wild type in male and female CBS-/+ animals respectively
GENERAL STABILITY
ORGANISM
UNIPROT
LITERATURE
binding of S-adenosyl-L-methionine stabilizes cystathionine beta-synthase against degradation. Under pathological conditions with reduced levels of S-adenosyl-L-methionine (mouse model for chronic steatohepatitis), level of cystathionine beta-synthase is diminished. This decrease in cystathionine beta-synthase level correlates with reduced glutathione that is, in turn, associated with increased vulnerability to oxidative stress. Posttranslational regulation of cystathionine beta-synthase stability by S-adenosyl-L-methionine provides a mechanism for achieving coordinate changes in cellular methylation and antioxidant status that is observed in a number of disease states
-
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
in hemizygous (+/-) CBS knockout mice the remaining CBS monoallele is up-regulated in mice when fed a taurine-deficient diet to produce additional CBS mRNA
-
mice at the unusual age of 28 months even have a higher hippocampal enzyme expression than young mice
-
treatment with homocysteine upregulates cystathionine gamma lyase CSE but downregulates CBS whereas Na2S or H2S-donoer GYY4137 downregulates CSE but upregulates CBS in a dose-dependent manner. In the homocysteine-treated cardiomyocytes, CBS and miR-133a are downregulated and hypertrophy is induced
-
APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
medicine
medicine
-
a deficiency of cystathionine beta-synthase causes various neurodevelopmental defects which result in complex neuropathological features associated with abnormal homocysteine metabolism, and also suggest that radial glia/astrocyte lineage cells might be a new therapeutic target for preventing and treating them
medicine
-
treatment with homocysteine upregulates cystathionine gamma lyase CSE but downregulates CBS whereas Na2S or H2S-donoer GYY4137 downregulates CSE but upregulates CBS in a dose-dependent manner. In the homocysteine-treated cardiomyocytes, CBS and miR-133a are downregulated and hypertrophy is inducedIn vivo studies using CBS+/- mice, a model for hyperhomocysteinemia, and sibling CBS+/+ control mice revealed that deficiency of CBS upregulates cardiac CSE
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Enokido, Y.; Suzuki, E.; Iwasawa, K.; Namekata, K.; Okazawa, H.; Kimura, H.
Cystathionine beta-synthase, a key enzyme for homocysteine metabolism, is preferentially expressed in the radial glia/astrocyte lineage of developing mouse CNS
FASEB J.
19
1854-1856
2005
Mus musculus
Pong, W.W.; Stouracova, R.; Frank, N.; Kraus, J.P.; Eldred, W.D.
Comparative localization of cystathionine beta-synthase and cystathionine gamma-lyase in retina: differences between amphibians and mammals
J. Comp. Neurol.
505
158-165
2007
Ambystoma tigrinum, Mus musculus
Vitvitsky, V.; Prudova, A.; Stabler, S.; Dayal, S.; Lentz, S.R.; Banerjee, R.
Testosterone regulation of renal cystathionine beta-synthase: implications for sex-dependent differences in plasma homocysteine levels
Am. J. Physiol. Renal Physiol.
293
F594-F600
2007
Cavia porcellus, Oryctolagus cuniculus, Homo sapiens, Mesocricetus auratus, Mus musculus, Rattus norvegicus
Liang, R.; Yu, W.; Du, J.; Yang, L.; Shang, M.; Guo, J.
Localization of cystathionine beta synthase in mice ovaries and its expression profile during follicular development
Chin. Med. J.
119
1877-1883
2006
Mus musculus
Hamelet, J.; Ait-Yahya-Graison, E.; Matulewicz, E.; Noll, C.; Badel-Chagnon, A.; Camproux, A.C.; Demuth, K.; Paul, J.L.; Delabar, J.M.; Janel, N.
Homocysteine threshold value based on cystathionine beta synthase and paraoxonase 1 activities in mice
Eur. J. Clin. Invest.
37
933-938
2007
Mus musculus
Prudova, A.; Bauman, Z.; Braun, A.; Vitvitsky, V.; Lu, S.C.; Banerjee, R.
S-adenosylmethionine stabilizes cystathionine beta-synthase and modulates redox capacity
Proc. Natl. Acad. Sci. USA
103
6489-6494
2006
Homo sapiens, Mus musculus
Liang, R.; Yu, W.D.; Du, J.B.; Yang, L.J.; Yang, J.J.; Xu, J.; Shang, M.; Guo, J.Z.
Cystathionine beta synthase participates in murine oocyte maturation mediated by homocysteine
Reprod. Toxicol.
24
89-96
2007
Mus musculus (Q91WT9), Mus musculus
Shintani, T.; Iwabuchi, T.; Soga, T.; Kato, Y.; Yamamoto, T.; Takano, N.; Hishiki, T.; Ueno, Y.; Ikeda, S.; Sakuragawa, T.; Ishikawa, K.; Goda, N.; Kitagawa, Y.; Kajimura, M.; Matsumoto, K.; Suematsu, M.
Cystathionine beta-synthase as a carbon monoxide-sensitive regulator of bile excretion
Hepatology
49
141-150
2009
Mus musculus, Rattus norvegicus
Ganapathy, P.S.; Moister, B.; Roon, P.; Mysona, B.A.; Duplantier, J.; Dun, Y.; Moister, T.K.; Farley, M.J.; Prasad, P.D.; Liu, K.; Smith, S.B.
Endogenous elevation of homocysteine induces retinal neuron death in the cystathionine-beta-synthase mutant mouse
Invest. Ophthalmol. Vis. Sci.
50
4460-4470
2009
Mus musculus
Chao, W.H.; Reynolds, R.D.
Taurine-deficient diet up-regulated cystathionine beta-synthase monoallele in hemizygous cystathionine beta-synthase knockout mice
Nutr. Res.
29
794-801
2009
Mus musculus
Jensen, K.K.; Geoghagen, N.S.; Jin, L.; Holt, T.G.; Luo, Q.; Malkowitz, L.; Ni, W.; Quan, S.; Waters, M.G.; Zhang, A.; Zhou, H.H.; Cheng, K.; Luo, M.J.
Pharmacological activation and genetic manipulation of cystathionine beta-synthase alter circulating levels of homocysteine and hydrogen sulfide in mice
Eur. J. Pharmacol.
650
86-93
2011
Homo sapiens, Mus musculus, Rattus norvegicus
Bruintjes, J.J.; Henning, R.H.; Douwenga, W.; van der Zee, E.A.
Hippocampal cystathionine beta synthase in young and aged mice
Neurosci. Lett.
563
135-139
2014
Mus musculus
Hirata, Y.; Funato, Y.; Takano, Y.; Miki, H.
Mg2+-dependent interactions of ATP with the cystathionine-beta-synthase (CBS) domains of a magnesium transporter
J. Biol. Chem.
289
14731-14739
2014
Mus musculus (Q3TWN3)
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