cystathionin-beta-synthasa [Cystathionine beta-Synthase]

tematický
49
Termíny

cystathionin-beta-syntáza
cystationin-beta-syntáza
cystationinsyntetáza

 

beta-Thionase
Cystathionine Synthetase
Serine Sulfhydrase

Perzistentní odkaz   https://www.medvik.cz/link/D003541
Definice

Multifunkční pyridoxalfosfátový enzym. Ve druhé fázi biosyntézy cysteinu katalyzuje reakci homocysteinu se serinem za vzniku cystathioninu s vyloučením vody. Deficit tohoto enzymu vede k hyperhomocysteinemii a homocysteinurii. EC 4.2.1.22.

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.

Anotace
/defic: consider also HOMOCYSTINURIA or HYPERHOMOCYSTEINEMIA
DUI
D003541 MeSH Prohlížeč
CUI
M0005533
CAS
L-Serine hydro-lyase (adding homocysteine)
Předchozí užití
Homocysteine (1973-1974); Hydro-Lyases (1973-1974); Serine (1973-1974)
Historická pozn.
1991(1975)
Veřejná pozn.
1991; see HYDRO-LYASES 1975-1990

D Chemikálie a léčiva
D08.811 enzymy 1 547
D08.811.520 lyasy 22
D08.811.520.241.300 dehydratasy 32
D08.811.520.241.300.050 akonitáthydratasa 8
D08.811.520.241.300.150 karboanhydrasy 52
D08.811.520.241.300.200 cystathionin-beta-synthasa 49
D08.811.520.241.300.250 enoyl-CoA-hydratasa 2
D08.811.520.241.300.275 enoyl-CoA-hydratáza 2
D08.811.520.241.300.287 syntázy mastných kyselin 13
D08.811.520.241.300.300 fumarasa 26
D08.811.520.241.300.500 fosfopyruváthydratasa 51
D08.811.520.241.300.550 porfobilinogensynthasa 10
D08.811.520.241.300.600 prefenátdehydratasa
D08.811.520.241.300.650 propandioldehydratasa
D08.811.520.241.300.850 tryptofansynthasa 2
D08.811.520.241.300.900 urokanasa
D08.811.520.241.300.950 uroporfyrinogen-III-synthetasa 1
D09 sacharidy 723
D10 lipidy 2 524