DB code: M00163

CATH domain -.-.-.- :
-.-.-.- :
3.-.-.- :
2.-.-.- :
2.40.50.100 : OB fold (Dihydrolipoamide Acetyltransferase, E2P)
3.50.50.60 : FAD/NAD(P)-binding domain Catalytic domain
3.50.50.60 : FAD/NAD(P)-binding domain
3.30.390.30 : Enolase-like; domain 1 Catalytic domain
E.C. 1.4.4.2 2.1.2.10 1.8.1.4
CSA
M-CSA
MACiE

CATH domain Related DB codes (homologues)
2.40.50.100 : OB fold (Dihydrolipoamide Acetyltransferase, E2P) M00222 M00145 M00188 M00189 T00223 M00190 M00191 M00208
3.30.390.30 : Enolase-like; domain 1 T00017 T00213 T00233 T00242
3.50.50.60 : FAD/NAD(P)-binding domain D00015 D00041 D00042 D00045 D00064 D00071 T00004 T00015 T00017 T00025 T00211 T00213 T00233 T00242

Uniprot Enzyme Name
UniprotKB Protein name Synonyms RefSeq Pfam
P49095 Glycine dehydrogenase [decarboxylating], mitochondrial
EC 1.4.4.2
Glycine decarboxylase
Glycine decarboxylase complex subunit P
Glycine cleavage system P-protein
NP_013914.1 (Protein)
NM_001182695.1 (DNA/RNA sequence)
PF02347 (GDC-P)
[Graphical View]
P26969 Glycine dehydrogenase [decarboxylating], mitochondrial
EC 1.4.4.2
Glycine decarboxylase
Glycine cleavage system P-protein
PF02347 (GDC-P)
[Graphical View]
P23378 Glycine dehydrogenase [decarboxylating], mitochondrial
EC 1.4.4.2
Glycine decarboxylase
Glycine cleavage system P-protein
NP_000161.2 (Protein)
NM_000170.2 (DNA/RNA sequence)
PF01212 (Beta_elim_lyase)
PF02347 (GDC-P)
[Graphical View]
P48015 Aminomethyltransferase, mitochondrial
EC 2.1.2.10
Glycine decarboxylase complex subunit T
Glycine cleavage system T protein
GCVT
NP_010302.1 (Protein)
NM_001180327.1 (DNA/RNA sequence)
PF01571 (GCV_T)
PF08669 (GCV_T_C)
[Graphical View]
P49364 Aminomethyltransferase, mitochondrial
EC 2.1.2.10
Glycine cleavage system T protein
GCVT
PF01571 (GCV_T)
PF08669 (GCV_T_C)
[Graphical View]
P48728 Aminomethyltransferase, mitochondrial
EC 2.1.2.10
Glycine cleavage system T protein
GCVT
NP_000472.2 (Protein)
NM_000481.3 (DNA/RNA sequence)
NP_001158182.1 (Protein)
NM_001164710.1 (DNA/RNA sequence)
NP_001158183.1 (Protein)
NM_001164711.1 (DNA/RNA sequence)
NP_001158184.1 (Protein)
NM_001164712.1 (DNA/RNA sequence)
PF01571 (GCV_T)
PF08669 (GCV_T_C)
[Graphical View]
P39726 Glycine cleavage system H protein, mitochondrial
Glycine decarboxylase complex subunit H
NP_009355.3 (Protein)
NM_001178189.1 (DNA/RNA sequence)
PF01597 (GCV_H)
[Graphical View]
P16048 Glycine cleavage system H protein, mitochondrial
None PF01597 (GCV_H)
[Graphical View]
P23434 Glycine cleavage system H protein, mitochondrial
None NP_004474.2 (Protein)
NM_004483.4 (DNA/RNA sequence)
PF01597 (GCV_H)
[Graphical View]
P09624 Dihydrolipoyl dehydrogenase, mitochondrial
EC 1.8.1.4
Dihydrolipoamide dehydrogenase
Lipoamide dehydrogenase component of pyruvate dehydrogenase complex
Pyruvate dehydrogenase complex E3 component
Glycine decarboxylase complex subunit L
NP_116635.1 (Protein)
NM_001179948.1 (DNA/RNA sequence)
PF00070 (Pyr_redox)
PF07992 (Pyr_redox_2)
PF02852 (Pyr_redox_dim)
[Graphical View]
P31023 Dihydrolipoyl dehydrogenase, mitochondrial
EC 1.8.1.4
Dihydrolipoamide dehydrogenase
Pyruvate dehydrogenase complex E3 subunit
PDC-E3
E3
Glycine cleavage system L protein
PF00070 (Pyr_redox)
PF07992 (Pyr_redox_2)
PF02852 (Pyr_redox_dim)
[Graphical View]
P09622 Dihydrolipoyl dehydrogenase, mitochondrial
EC 1.8.1.4
Dihydrolipoamide dehydrogenase
Glycine cleavage system L protein
NP_000099.2 (Protein)
NM_000108.3 (DNA/RNA sequence)
PF00070 (Pyr_redox)
PF07992 (Pyr_redox_2)
PF02852 (Pyr_redox_dim)
[Graphical View]

KEGG enzyme name
glycine dehydrogenase (decarboxylating)
(EC 1.4.4.2 )
P-protein
(EC 1.4.4.2 )
glycine decarboxylase
(EC 1.4.4.2 )
glycine-cleavage complex
(EC 1.4.4.2 )
glycine:lipoylprotein oxidoreductase (decarboxylating andacceptor-aminomethylating)
(EC 1.4.4.2 )
protein P1
(EC 1.4.4.2 )
aminomethyltransferase
(EC 2.1.2.10 )
S-aminomethyldihydrolipoylprotein:(6S)-tetrahydrofolateaminomethyltransferase (ammonia-forming)
(EC 2.1.2.10 )
T-protein
(EC 2.1.2.10 )
glycine synthase
(EC 2.1.2.10 )
tetrahydrofolate aminomethyltransferase
(EC 2.1.2.10 )
[protein]-8-S-aminomethyldihydrolipoyllysine:tetrahydrofolateaminomethyltransferase (ammonia-forming)
(EC 2.1.2.10 )
dihydrolipoyl dehydrogenase
(EC 1.8.1.4 )
LDP-Glc
(EC 1.8.1.4 )
LDP-Val
(EC 1.8.1.4 )
dehydrolipoate dehydrogenase
(EC 1.8.1.4 )
diaphorase
(EC 1.8.1.4 )
dihydrolipoamide dehydrogenase
(EC 1.8.1.4 )
dihydrolipoamide:NAD+ oxidoreductase
(EC 1.8.1.4 )
dihydrolipoic dehydrogenase
(EC 1.8.1.4 )
dihydrothioctic dehydrogenase
(EC 1.8.1.4 )
lipoamide dehydrogenase (NADH)
(EC 1.8.1.4 )
lipoamide oxidoreductase (NADH)
(EC 1.8.1.4 )
lipoamide reductase
(EC 1.8.1.4 )
lipoamide reductase (NADH)
(EC 1.8.1.4 )
lipoate dehydrogenase
(EC 1.8.1.4 )
lipoic acid dehydrogenase
(EC 1.8.1.4 )
lipoyl dehydrogenase
(EC 1.8.1.4 )
protein-6-N-(dihydrolipoyl)lysine:NAD+ oxidoreductase
(EC 1.8.1.4 )

UniprotKB: Accession Number Entry name Activity Subunit Subcellular location Cofactor
P49095 GCSP_YEAST Glycine + H-protein-lipoyllysine = H-protein- S-aminomethyldihydrolipoyllysine + CO(2). Component of the glycine decarboxylase complex (GDC), which is composed of four proteins: P, T, L and H. Mitochondrion (By similarity). Pyridoxal phosphate (By similarity).
P26969 GCSP_PEA Glycine + H-protein-lipoyllysine = H-protein- S-aminomethyldihydrolipoyllysine + CO(2). Homodimer. The glycine cleavage system is composed of four proteins: P, T, L and H. Mitochondrion. Pyridoxal phosphate.
P23378 GCSP_HUMAN Glycine + H-protein-lipoyllysine = H-protein- S-aminomethyldihydrolipoyllysine + CO(2). Homodimer. The glycine cleavage system is composed of four proteins: P, T, L and H. Mitochondrion. Pyridoxal phosphate.
P48015 GCST_YEAST [Protein]-S(8)-aminomethyldihydrolipoyllysine + tetrahydrofolate = [protein]-dihydrolipoyllysine + 5,10- methylenetetrahydrofolate + NH(3). Component of the glycine decarboxylase complex (GDC), which is composed of four proteins: P, T, L and H. Mitochondrion.
P49364 GCST_PEA [Protein]-S(8)-aminomethyldihydrolipoyllysine + tetrahydrofolate = [protein]-dihydrolipoyllysine + 5,10- methylenetetrahydrofolate + NH(3). The glycine cleavage system is composed of four proteins: P, T, L and H. Mitochondrion.
P48728 GCST_HUMAN [Protein]-S(8)-aminomethyldihydrolipoyllysine + tetrahydrofolate = [protein]-dihydrolipoyllysine + 5,10- methylenetetrahydrofolate + NH(3). The glycine cleavage system is composed of four proteins: P, T, L and H. Mitochondrion.
P39726 GCSH_YEAST Component of the glycine decarboxylase complex (GDC), which is composed of four proteins: P, T, L and H. Mitochondrion. Binds 1 lipoyl cofactor covalently (By similarity).
P16048 GCSH_PEA The glycine cleavage system is composed of four proteins: P, T, L and H. Mitochondrion. Binds 1 lipoyl cofactor covalently.
P23434 GCSH_HUMAN The glycine cleavage system is composed of four proteins: P, T, L and H. Mitochondrion. Binds 1 lipoyl cofactor covalently.
P09624 DLDH_YEAST Protein N(6)-(dihydrolipoyl)lysine + NAD(+) = protein N(6)-(lipoyl)lysine + NADH. LPD1 is a homodimer. Eukaryotic pyruvate dehydrogenase (PDH) complexes are organized as a core consisting of the oligomeric dihydrolipoamide acetyl-transferase (E2), around which are arranged multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide dehydrogenase (E3) and protein X (E3BP) bound by non-covalent bonds. LPD1 is a component of the glycine decarboxylase complex (GDC), which is composed of four proteins: P, T, L and H. Mitochondrion matrix. Binds 1 FAD per subunit (By similarity).
P31023 DLDH_PEA Protein N(6)-(dihydrolipoyl)lysine + NAD(+) = protein N(6)-(lipoyl)lysine + NADH. Homodimer. Mitochondrion matrix. Binds 1 FAD per subunit.
P09622 DLDH_HUMAN Protein N(6)-(dihydrolipoyl)lysine + NAD(+) = protein N(6)-(lipoyl)lysine + NADH. Homodimer. Eukaryotic pyruvate dehydrogenase complexes are organized about a core consisting of the oligomeric dihydrolipoamide acetyl-transferase, around which are arranged multiple copies of pyruvate dehydrogenase, dihydrolipoamide dehydrogenase and protein X bound by non-covalent bonds. Mitochondrion matrix. Binds 1 FAD per subunit (By similarity).

KEGG Pathways
Map code Pathways E.C.
MAP00010 Glycolysis / Gluconeogenesis 1.8.1.4
MAP00020 Citrate cycle (TCA cycle) 1.8.1.4
MAP00260 Glycine, serine and threonine metabolism 1.4.4.2 2.1.2.10 1.8.1.4
MAP00280 Valine, leucine and isoleucine degradation 1.8.1.4
MAP00620 Pyruvate metabolism 1.8.1.4
MAP00670 One carbon pool by folate 2.1.2.10
MAP00910 Nitrogen metabolism 2.1.2.10

Compound table
Cofactors Substrates Products Intermediates
KEGG-id C00018 C00016 C00037 C02051 C01242 C00101 C02972 C00003 C01242 C00011 C02972 C00143 C00014 C02051 C00004 C00080
E.C. 1.4.4.2
1.8.1.4
1.4.4.2
1.4.4.2
2.1.2.10
2.1.2.10
1.8.1.4
1.8.1.4
1.4.4.2
1.4.4.2
2.1.2.10
2.1.2.10
2.1.2.10
1.8.1.4
1.8.1.4
1.8.1.4
Compound Pyridoxal phosphate FAD Glycine Lipoylprotein S-Aminomethyldihydrolipoylprotein Tetrahydrofolate Dihydrolipoylprotein NAD+ S-Aminomethyldihydrolipoylprotein CO2 Dihydrolipoylprotein 5,10-methylenetetrahydrofolate NH3 Lipoylprotein NADH H+
Type aromatic ring (with nitrogen atoms),phosphate group/phosphate ion amide group,amine group,aromatic ring (only carbon atom),aromatic ring (with nitrogen atoms),carbohydrate,nucleotide amino acids disulfide bond,lipid,peptide/protein amine group,lipid,peptide/protein,sulfhydryl group,sulfide group amino acids,amide group,amine group,aromatic ring (only carbon atom),aromatic ring (with nitrogen atoms),carboxyl group carbohydrate,lipid,peptide/protein,sulfhydryl group amide group,amine group,nucleotide amine group,lipid,peptide/protein,sulfhydryl group,sulfide group others carbohydrate,lipid,peptide/protein,sulfhydryl group amino acids,amide group,amine group,aromatic ring (only carbon atom),aromatic ring (with nitrogen atoms),carboxyl group amine group,organic ion disulfide bond,lipid,peptide/protein amide group,amine group,nucleotide others
ChEBI 18405
 18405
 		16238
 16238
 		15428
 57305
 15428
 57305
 		15635
 20506
 15635
 20506
 		15846
 15846
 		16526
 16526
 		16134
 16134
 		16908
 16908
 		15378
 15378
PubChem 1051
 1051
 		643975
 643975
 		5257127
 750
 5257127
 750
 		5460413
 91443
 5460413
 91443
 		5893
 5893
 		280
 280
 		439175
 439175
 		222
 222
 		439153
 439153
 		1038
 1038
1wsrA01 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsrB01 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsvA01 Unbound Unbound Unbound Unbound Unbound Analogue:THH Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsvB01 Unbound Unbound Unbound Unbound Unbound Analogue:THH Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsrA02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsrB02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsvA02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1wsvB02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxmA Unbound Unbound Unbound Unbound Unbound Unbound Bound:RED Unbound Unbound Unbound Bound:RED Unbound Unbound Unbound Unbound
1dxmB Unbound Unbound Unbound Unbound Unbound Unbound Bound:RED Unbound Unbound Unbound Bound:RED Unbound Unbound Unbound Unbound
1hpcA Unbound Unbound Unbound Bound:LPA Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:LPA Unbound
1hpcB Unbound Unbound Unbound Bound:LPA Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:LPA Unbound
1htpA Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1jehA01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1jehB01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1v59A01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1v59B01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlA01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlB01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlC01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlD01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcA01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcB01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcC01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcD01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcE01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcF01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcG01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcH01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdA01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdB01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdC01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdD01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdE01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdF01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdG01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdH01 Unbound Bound:FAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1jehA02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1jehB02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1v59A02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1v59B02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlA02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlB02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlC02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlD02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcA02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcB02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcC02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcD02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcE02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcF02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcG02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcH02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Analogue:NAD Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdA02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdB02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdC02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdD02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdE02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdF02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdG02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1zmdH02 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Bound:NAI
1jehA03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1jehB03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1v59A03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1v59B03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlA03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlB03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlC03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1dxlD03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcA03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcB03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcC03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcD03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcE03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcF03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcG03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmcH03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdA03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdB03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdC03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdD03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdE03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdF03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdG03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound
1zmdH03 Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound Unbound

Reference for Active-site residues
resource references E.C.
literature [20] & see T00017

Active-site residues
PDB Catalytic residues Cofactor-binding residues Modified residues Main-chain involved in catalysis Comment
1wsrA01 ASP 101
1wsrB01 ASP 101
1wsvA01 ASP 101
1wsvB01 ASP 101
1wsrA02
1wsrB02
1wsvA02
1wsvB02
1dxmA LYS 63(lipoyl cofactor binding)
1dxmB LYS 63(lipoyl cofactor binding)
1hpcA LYS 63(lipoyl cofactor binding)
1hpcB LYS 63(lipoyl cofactor binding)
1htpA LYS 63(lipoyl cofactor binding)
1jehA01 TYR 18;CYS 44;CYS 49
1jehB01 TYR 18;CYS 44;CYS 49
1v59A01 TYR 18;CYS 44;CYS 49
1v59B01 TYR 18;CYS 44;CYS 49
1dxlA01 TYR 19;CYS 45;CYS 50
1dxlB01 TYR 19;CYS 45;CYS 50
1dxlC01 TYR 19;CYS 45;CYS 50
1dxlD01 TYR 19;CYS 45;CYS 50
1zmcA01 TYR 19;CYS 45;CYS 50
1zmcB01 TYR 19;CYS 45;CYS 50
1zmcC01 TYR 19;CYS 45;CYS 50
1zmcD01 TYR 19;CYS 45;CYS 50
1zmcE01 TYR 19;CYS 45;CYS 50
1zmcF01 TYR 19;CYS 45;CYS 50
1zmcG01 TYR 19;CYS 45;CYS 50
1zmcH01 TYR 19;CYS 45;CYS 50
1zmdA01 TYR 19;CYS 45;CYS 50
1zmdB01 TYR 19;CYS 45;CYS 50
1zmdC01 TYR 19;CYS 45;CYS 50
1zmdD01 TYR 19;CYS 45;CYS 50
1zmdE01 TYR 19;CYS 45;CYS 50
1zmdF01 TYR 19;CYS 45;CYS 50
1zmdG01 TYR 19;CYS 45;CYS 50
1zmdH01 TYR 19;CYS 45;CYS 50
1jehA02
1jehB02
1v59A02
1v59B02
1dxlA02
1dxlB02
1dxlC02
1dxlD02
1zmcA02
1zmcB02
1zmcC02
1zmcD02
1zmcE02
1zmcF02
1zmcG02
1zmcH02
1zmdA02
1zmdB02
1zmdC02
1zmdD02
1zmdE02
1zmdF02
1zmdG02
1zmdH02
1jehA03 HIS 457;HIS 477
1jehB03 HIS 457;HIS 477
1v59A03 HIS 457;HIS 477
1v59B03 HIS 457;HIS 477
1dxlA03 HIS 449;HIS 469
1dxlB03 HIS 449;HIS 469
1dxlC03 HIS 449;HIS 469
1dxlD03 HIS 449;HIS 469
1zmcA03 HIS 452;ASN 473
1zmcB03 HIS 452;ASN 473
1zmcC03 HIS 452;ASN 473
1zmcD03 HIS 452;ASN 473
1zmcE03 HIS 452;ASN 473
1zmcF03 HIS 452;ASN 473
1zmcG03 HIS 452;ASN 473
1zmcH03 HIS 452;ASN 473
1zmdA03 HIS 452;ASN 473
1zmdB03 HIS 452;ASN 473
1zmdC03 HIS 452;ASN 473
1zmdD03 HIS 452;ASN 473
1zmdE03 HIS 452;ASN 473
1zmdF03 HIS 452;ASN 473
1zmdG03 HIS 452;ASN 473
1zmdH03 HIS 452;ASN 473

References for Catalytic Mechanism
References Sections No. of steps in catalysis
[20]
Fig.3, p.1148-1151

References
[1]
Resource
Comments
Medline ID
PubMed ID 6750353
Journal Mol Cell Biochem
Year 1982
Volume 45
Pages 137-49
Authors Kikuchi G, Hiraga K
Title The mitochondrial glycine cleavage system. Unique features of the glycine decarboxylation.
Related PDB
Related UniProtKB
[2]
Resource
Comments
Medline ID
PubMed ID 1856858
Journal J Mol Biol
Year 1991
Volume 220
Pages 223-4
Authors Sieker L, Cohen-Addad C, Neuburger M, Douce R
Title Crystallographic data for H-protein from the glycine decarboxylase complex.
Related PDB
Related UniProtKB
[3]
Resource
Comments X-RAY CRYSTALLOGRAPHY (2.6 ANGSTROMS)
Medline ID 94255425
PubMed ID 8197146
Journal Proc Natl Acad Sci U S A
Year 1994
Volume 91
Pages 4850-3
Authors Pares S, Cohen-Addad C, Sieker L, Neuburger M, Douce R
Title X-ray structure determination at 2.6-A resolution of a lipoate-containing protein: the H-protein of the glycine decarboxylase complex from pea leaves.
Related PDB
Related UniProtKB P16048
[4]
Resource
Comments X-ray crystallography
Medline ID
PubMed ID
Journal Acta Crystallogr D Biol Crystallogr
Year 1995
Volume 51
Pages 1041-51
Authors Pares S, Cohenaddad C, Sieker LC, Neuburger M, Douce R
Title Refined structures at 2 and 2.2 A resolution of two forms of the H-protein, a lipoamide-containing protein of the glycine decarboxylase complex.
Related PDB 1hpc
Related UniProtKB
[5]
Resource
Comments X-ray crystallography
Medline ID
PubMed ID 7719855
Journal Nat Struct Biol
Year 1995
Volume 2
Pages 63-8
Authors Cohen-Addad C, Pares S, Sieker L, Neuburger M, Douce R
Title The lipoamide arm in the glycine decarboxylase complex is not freely swinging.
Related PDB 1htp
Related UniProtKB
[6]
Resource
Comments
Medline ID
PubMed ID 8830251
Journal Mol Microbiol
Year 1996
Volume 19
Pages 611-23
Authors Sinclair DA, Hong SP, Dawes IW
Title Specific induction by glycine of the gene for the P-subunit of glycine decarboxylase from Saccharomyces cerevisiae.
Related PDB
Related UniProtKB
[7]
Resource
Comments
Medline ID
PubMed ID 9479445
Journal Biochimie
Year 1997
Volume 79
Pages 637-43
Authors Cohen-Addad C, Faure M, Neuburger M, Ober R, Sieker L, Bourguignon J, Macherel D, Douce R
Title Structural studies of the glycine decarboxylase complex from pea leaf mitochondria.
Related PDB
Related UniProtKB
[8]
Resource
Comments
Medline ID
PubMed ID 9538259
Journal J Biochem (Tokyo)
Year 1998
Volume 123
Pages 668-74
Authors Toyoda T, Suzuki K, Sekiguchi T, Reed LJ, Takenaka A
Title Crystal structure of eucaryotic E3, lipoamide dehydrogenase from yeast.
Related PDB 1jeh
Related UniProtKB
[9]
Resource
Comments
Medline ID
PubMed ID 10387079
Journal Biochemistry
Year 1999
Volume 38
Pages 8334-46
Authors Guilhaudis L, Simorre JP, Blackledge M, Neuburger M, Bourguignon J, Douce R, Marion D, Gans P
Title Investigation of the local structure and dynamics of the H subunit of the mitochondrial glycine decarboxylase using heteronuclear NMR spectroscopy.
Related PDB
Related UniProtKB
[10]
Resource
Comments
Medline ID
PubMed ID 10375564
Journal Curr Opin Plant Biol
Year 1999
Volume 2
Pages 214-22
Authors Douce R, Neuburger M
Title Biochemical dissection of photorespiration.
Related PDB
Related UniProtKB
[11]
Resource
Comments
Medline ID
PubMed ID 10473591
Journal J Biol Chem
Year 1999
Volume 274
Pages 26344-52
Authors Gueguen V, Macherel D, Neuburger M, Pierre CS, Jaquinod M, Gans P, Douce R, Bourguignon J
Title Structural and functional characterization of H protein mutants of the glycine decarboxylase complex.
Related PDB
Related UniProtKB
[12]
Resource
Comments
Medline ID
PubMed ID 10605092
Journal J Biomol NMR
Year 1999
Volume 15
Pages 185-6
Authors Guilhaudis L, Simorre JP, Bouchayer E, Neuburger M, Bourguignon J, Douce R, Marion D, Gans P
Title Backbone and sequence-specific assignment of three forms of the lipoate-containing H-protein of the glycine decarboxylase complex.
Related PDB
Related UniProtKB
[13]
Resource
Comments
Medline ID
PubMed ID 10398369
Journal Proteins
Year 1999
Volume 36
Pages 228-37
Authors Roche O, Hinsen K, Field MJ
Title Theoretical study of the conformation of the H-protein lipoamide arm as a function of its terminal group.
Related PDB
Related UniProtKB
[14]
Resource
Comments
Medline ID
PubMed ID 10757974
Journal Biochemistry
Year 2000
Volume 39
Pages 4259-66
Authors Guilhaudis L, Simorre JP, Blackledge M, Marion D, Gans P, Neuburger M, Douce R
Title Combined structural and biochemical analysis of the H-T complex in the glycine decarboxylase cycle: evidence for a destabilization mechanism of the H-protein.
Related PDB
Related UniProtKB
[15]
Resource
Comments X-ray crystallography
Medline ID
PubMed ID 10806386
Journal Eur J Biochem
Year 2000
Volume 267
Pages 2890-8
Authors Faure M, Bourguignon J, Neuburger M, MacHerel D, Sieker L, Ober R, Kahn R, Cohen-Addad C, Douce R
Title Interaction between the lipoamide-containing H-protein and the lipoamide dehydrogenase (L-protein) of the glycine decarboxylase multienzyme system 2. Crystal structures of H- and L-proteins.
Related PDB 1dxm 1dxl
Related UniProtKB
[16]
Resource
Comments
Medline ID
PubMed ID 10806385
Journal Eur J Biochem
Year 2000
Volume 267
Pages 2882-9
Authors Neuburger M, Polidori AM, Pietre E, Faure M, Jourdain A, Bourguignon J, Pucci B, Douce R
Title Interaction between the lipoamide-containing H-protein and the lipoamide dehydrogenase (L-protein) of the glycine decarboxylase multienzyme system. 1. Biochemical studies.
Related PDB
Related UniProtKB
[17]
Resource
Comments
Medline ID
PubMed ID 11599027
Journal Proteins
Year 2001
Volume 45
Pages 237-40
Authors Roche O, Field MJ
Title Theoretical study of the conformation of the lipoamide arm in a mutant H protein.
Related PDB
Related UniProtKB
[18]
Resource
Comments
Medline ID
PubMed ID 11286922
Journal Trends Plant Sci
Year 2001
Volume 6
Pages 167-76
Authors Douce R, Bourguignon J, Neuburger M, Rebeille F
Title The glycine decarboxylase system: a fascinating complex.
Related PDB
Related UniProtKB
[19]
Resource
Comments
Medline ID
PubMed ID 15946682
Journal J Mol Biol
Year 2005
Volume 350
Pages 543-52
Authors Brautigam CA, Chuang JL, Tomchick DR, Machius M, Chuang DT
Title Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations.
Related PDB 1zmc 1zmd
Related UniProtKB
[20]
Resource
Comments
Medline ID
PubMed ID 16051266
Journal J Mol Biol
Year 2005
Volume 351
Pages 1146-59
Authors Okamura-Ikeda K, Hosaka H, Yoshimura M, Yamashita E, Toma S, Nakagawa A, Fujiwara K, Motokawa Y, Taniguchi H
Title Crystal structure of human T-protein of glycine cleavage system at 2.0 A resolution and its implication for understanding non-ketotic hyperglycinemia.
Related PDB 1wsr 1wsv
Related UniProtKB

Comments
The glycine cleavage system, which catalyzes three distinct reactions (EC 1.4.4.2, 2.1.2.10, 1.8.1.4) is a protein comprex composed of the four proteins:
P chain is responsible for the cleavage of glycine (EC 1.4.4.2).
T chain is responsible for the transfer of aminomethyl group (EC 2.1.2.10).
H chain transports methylamine group from the P chain to the T chain, with the lysine residue (Lys63) covalently-bound to lipoyl group.
L chain is responsible for the oxidation of dihydrolipoyl group (EC 1.8.1.4). This enzyme is homologous to the counterpart enzyme (T00017 in EzCatDB).
Although the structures of T, H and L chain have been determined, those of the other chain have not been solved yet.
As for the reaction by T chain (EC 2.1.2.10), the two consecutive transfer reactions might be involved (see [20]).

Created Updated
2004-01-29 2009-03-13