1.4.4.2: glycine dehydrogenase (aminomethyl-transferring)
This is an abbreviated version!
For detailed information about glycine dehydrogenase (aminomethyl-transferring), go to the full flat file.
Word Map on EC 1.4.4.2
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1.4.4.2
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coil
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aneurysm
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guglielmi
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embolization
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endovascular
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artery
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measles
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occlusion
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cerebral
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intracranial
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hemagglutinin
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rupture
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neck
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dental
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subarachnoid
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hemorrhage
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photorespiratory
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angiographic
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photorespiration
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council
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distemper
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hyperglycinemia
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dentist
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lipoylation
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hydroxymethyltransferase
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polyomavirus
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angiogram
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t-proteins
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slam
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lipoic
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nonketotic
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rebleeding
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microcatheter
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morbillivirus
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vasospasm
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unruptured
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recanalization
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basilar
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polyoma
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ceria
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glycodeoxycholate
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practise
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procedure-related
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lipoamide
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profession
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flaveria
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lipoate
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panencephalitis
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medicine
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postal
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curriculum
- 1.4.4.2
-
coil
- aneurysm
-
guglielmi
-
embolization
-
endovascular
- artery
- measles
-
occlusion
- cerebral
-
intracranial
- hemagglutinin
-
rupture
- neck
-
dental
-
subarachnoid
- hemorrhage
-
photorespiratory
-
angiographic
-
photorespiration
-
council
- distemper
- hyperglycinemia
-
dentist
-
lipoylation
- hydroxymethyltransferase
- polyomavirus
-
angiogram
-
t-proteins
-
slam
-
lipoic
-
nonketotic
-
rebleeding
-
microcatheter
- morbillivirus
- vasospasm
-
unruptured
-
recanalization
-
basilar
-
polyoma
-
ceria
- glycodeoxycholate
-
practise
-
procedure-related
- lipoamide
-
profession
- flaveria
- lipoate
- panencephalitis
- medicine
-
postal
-
curriculum
Reaction
Synonyms
decarboxylase, glycine, GDC, GDCP, GLDC, GLDH, GLDP, Gly decarboxylase complex, Gly decarboxylase H1, glycine cleavage enzyme complex, glycine cleavage H protein, glycine cleavage system H protein 1, Glycine cleavage system P-protein, glycine decarboxylase, glycine decarboxylase (P-protein), glycine decarboxylase complex, glycine decarboxylase complex H, glycine decarboxylase P-protein, glycine dehydrogenase, glycine dehydrogenase (decarboxylating), glycine-cleavage complex, H protein, H-protein, H1 protein, H2 protein, L protein, More, P protein, P-protein, P-protein (glycine decarboxylase), P-subunit, Protein P1, T protein
ECTree
1.4.4.2 glycine dehydrogenase (aminomethyl-transferring)Advanced search results
results (
results (Substrates Products
Substrates Products on EC 1.4.4.2 - glycine dehydrogenase (aminomethyl-transferring)
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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
glycine + [glycine-cleavage complex H protein]-N6-lipoyl-L-lysine
[glycine-cleavage complex H protein]-S-aminomethyl-N6-dihydrolipoyl-L-lysine + CO2
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
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?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
-
?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
-
?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
-
?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
P-protein is the actual glycine-decarboxylating enzyme and uses pyridoxal 5'-phosphate as a cofactor. CO2 is released in the reaction and the residual aminomethyl group is bound to the oxidized lipoamide arm of H-protein
the methylene group is accepted by tetrahydrofolate to yield methylene tetrahydrofolate, a major cofactor in one-carbon metabolism
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?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
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?
glycine + H-protein-lipoyllysine
H-protein-S-aminomethyldihydrolipoyllysine + CO2
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-
-
-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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-
-
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?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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reaction is stimulated by lipoic acid which is a functional group of the H-protein
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?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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glycine decarboxylation catalyzed by P-protein alone is extremely low
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?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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-
-
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?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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-
-
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?
glycine + lipoylprotein
S-aminomethyldihydrolipoylprotein + CO2
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lipoyl protein: H-protein, lipoamide can also act as acceptor
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-
?
glycine + [glycine-cleavage complex H protein]-N6-lipoyl-L-lysine
[glycine-cleavage complex H protein]-S-aminomethyl-N6-dihydrolipoyl-L-lysine + CO2
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r
glycine + [glycine-cleavage complex H protein]-N6-lipoyl-L-lysine
[glycine-cleavage complex H protein]-S-aminomethyl-N6-dihydrolipoyl-L-lysine + CO2
reversible reaction
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r
additional information
?
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-
the GDC H-protein (GLDH) has no catalytic activity itself but interacts via its lipoyl arm one after the other with the three other GDC subunits, P-protein (EC 1.4.4.2), T-protein (EC 2.1.2.10), and L-protein (EC 1.8.1.4)
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?
additional information
?
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the GDC H-protein is a substrate for P-protein of glycine decarboxylase
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?
additional information
?
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P-protein also catalyzes exchange of carbonyl carbon of glycine with CO2, reaction greatly stimulated by addition of H protein
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?
additional information
?
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-
P-protein also catalyzes exchange of carbonyl carbon of glycine with CO2, reaction greatly stimulated by addition of H protein
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?
additional information
?
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the glycine cleavage system consists of 4 protein components: 1. P-protein is a pyridoxal containing protein: a Schiff base is formed between the hydroxyl group of the pyridoxal phosphate and the alpha-NH2 of glycine, the amino group and the alpha-carbon of the glycine are transferred to the lipoamide cofactor of the second enzyme of the complex the H-protein, the alpha-carbonyl group of glycine is lost as CO2, 2. H-protein, 3. T-protein: catalyzes the passage of alpha-carbon from lipoamide of H protein to tetrahydrofolate, alpha-NH2 from glycine is lost as NH4+, 4. L-protein: catalyzes oxidation of reduced lipoamide back to its original form with concomitant reduction of NAD+ to NADH
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?
additional information
?
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nonketotic hyperglycinaemia is an autosomal recessive disorder of glycine metabolism caused by a deficiency in the mitochondrial glycine cleavage enzyme. The majority of cases are caused by mutations in the P-protein
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?
additional information
?
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presence of excess pyridoxal 5'-phosphate is used in the assay mixture to facilitate the displacement of the quinonoid intermediate in the absence of an acceptor for the methylamine moiety (of decarboxylated glycine) to allow multiple enzyme turnovers. Mass spectrometry and 13C NMR spectroscopy are used to determine the products of glycine decarboxylation. The assay reaction mixtures are incubated for 1 week at pH 7.0, 25°C
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?
additional information
?
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-
presence of excess pyridoxal 5'-phosphate is used in the assay mixture to facilitate the displacement of the quinonoid intermediate in the absence of an acceptor for the methylamine moiety (of decarboxylated glycine) to allow multiple enzyme turnovers. Mass spectrometry and 13C NMR spectroscopy are used to determine the products of glycine decarboxylation. The assay reaction mixtures are incubated for 1 week at pH 7.0, 25°C
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-
?
additional information
?
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-
glycine decarboxylase does not occur as a complex of all four proteins, but L-protein takes part in both glycine decarboxylase as well as in glycine reductase reaction
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?
additional information
?
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P-protein also catalyzes exchange of carbonyl carbon of glycine with CO2, reaction greatly stimulated by addition of H protein
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?
additional information
?
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the enzyme is a component of the reversible glycine cleavage system, previously known as glycine synthase
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?
additional information
?
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in transgenic potato plants with an antisense reduction in P-protein of GDC, the decrease in photorespiratory decarboxylation is compensated for by an increase in respiratory decarboxylation in the light
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?
