4.2.1.22: cystathionine beta-synthase
This is an abbreviated version!
For detailed information about cystathionine beta-synthase, go to the full flat file.
Word Map on EC 4.2.1.22
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4.2.1.22
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h2s
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sulfide
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homocystinuria
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hyperhomocysteinemia
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artery
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spacer
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corticobasal
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candida
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mthfr
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carotid
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folate
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transsulfuration
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conidia
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cajal
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nahs
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dextrose
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methylenetetrahydrofolate
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bismuth
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hallucinations
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anamorphic
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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
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biodiversity
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hyaline
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chemoreceptor
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gasotransmitter
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3-mercaptopyruvate
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frontotemporal
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sulfurtransferase
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thromboembolic
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ascospore
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rinsed
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aminooxyacetic
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apraxia
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snrnps
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symptomless
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hydrosulfide
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marxianus
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visuospatial
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5,10-methylenetetrahydrofolate
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voxel-based
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appressoria
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naocl
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medicine
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diagnostics
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ascus
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phytopathological
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analysis
- 4.2.1.22
- h2s
- sulfide
- homocystinuria
- hyperhomocysteinemia
- artery
-
spacer
-
corticobasal
- candida
- mthfr
-
carotid
- folate
-
transsulfuration
- conidia
-
cajal
- nahs
- dextrose
- methylenetetrahydrofolate
-
bismuth
- hallucinations
-
anamorphic
- palsy
-
supranuclear
-
charles
-
remethylation
-
reisolated
-
conidiophore
-
gamma-lyase
-
biodiversity
-
hyaline
-
chemoreceptor
-
gasotransmitter
- 3-mercaptopyruvate
-
frontotemporal
- sulfurtransferase
-
thromboembolic
- ascospore
-
rinsed
-
aminooxyacetic
- apraxia
-
snrnps
-
symptomless
- hydrosulfide
- marxianus
-
visuospatial
- 5,10-methylenetetrahydrofolate
-
voxel-based
-
appressoria
- naocl
- medicine
- diagnostics
- ascus
-
phytopathological
- analysis
Reaction
Synonyms
Beta-thionase, CBS, CBS424, CDCP2, CNNM2, Cys4, CysB, cystathionine beta synthase, cystathionine beta-synthase, cystathionine beta-synthase domain-containing protein, cystathionine-beta-synthase, Cysteine synthase, EC 4.2.1.21, hCBS, Hemoprotein H-450, LbrM.17.0230, Methylcysteine synthase, osmoprotectant transporter OpuC, PF1953, PH0267, Serine sulfhydrase, Serine sulfhydrylase, Serine sulphhydrase, TA0289, TM0935, TV1335, yCBS, ytCBS
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Natural Substrates Products
Natural Substrates Products on EC 4.2.1.22 - cystathionine beta-synthase
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REACTION DIAGRAM
L-Serine + homocysteine
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L-Serine + homocysteine
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reduced activity of EC 4.2.1.22 in patients with homocystinuria due to mutations in the CBS gene
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L-Serine + homocysteine
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initial step in transsulfuration forming cysteine. Homocysteine is a relatively toxic amino acid, which levels have to be kept low
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L-serine + L-homocysteine
cystathionine + H2O
the enzyme participates in the process of oocyte maturation
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L-serine + L-homocysteine
L-cystathionine + H2O
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key enzyme involved in intracellular metabolism of homocysteine
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additional information
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elevated total plasma homocysteine is an independent risk factor in the development of vascular disease in humans. Elevating cystathionine beta-synthase level is an effective method to lower plasma homocysteine levels
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additional information
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the enzyme may have additional in vivo functions beyond its role as a homocysteine metabolizing enzyme
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additional information
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the overexpression of cystathionine beta-synthase may cause the developmental abnormality in cognition in Down's syndrome children and may lead to Alzheimer type of disease in Down's syndrom adults
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additional information
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the cystathionine beta-synthase variant c.844_845ins68 protects against CNS demyelination in X-linked adrenoleukodystrophy
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additional information
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the oxidation of CBS by dioxygen appears to proceed directly from the ferrous to the ferric state, presumably via an outer sphere electron transfer reaction
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additional information
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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
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additional information
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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
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additional information
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role for CBS as a mediator in interactions between oocyte and granulosa cells
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additional information
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cystathionine-beta-synthase domains in the ATP-binding component of OpuC are required for transporter function
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additional information
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plasma albumin cysteinylation is regulated by cystathionine beta-synthase
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additional information
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key enzyme in the trans-sulfuration pathway. Cystathionine beta-synthase may be an oxidative defense enzyme in the eye tissue, in particular in the segments of the eye where constant environmental oxidative stress is imposed
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