Gamma carboxylation, hypusine formation and arylsulfatase activation (Homo sapiens)

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13, 14, 30, 3613, 14, 30, 364, 6, 8, 18, 28...127, 31, 3513, 14, 30, 362, 1013, 14, 30, 3613, 14, 30, 3613, 14, 30, 3613, 14, 16, 30, 3621, 27, 355, 24pro-factor X [Golgilumen]cytosolpro-protein C [Golgilumen]factor X [Golgilumen]protein C [Golgilumen]DOHH:Fe++ [cytosol]pro-protein C[endoplasmicreticulum lumen]Golgi lumenpro-factor X[endoplasmicreticulum lumen]SUMF1:SUMF2[endoplasmicreticulum lumen]deoxyhypusinesynthase tetramer[cytosol]pro-protein C,uncarboxylated[endoplasmicreticulum lumen]endoplasmic reticulum lumenpro-factor X,uncarboxylated[endoplasmicreticulum lumen]pro-protein CSUMF1:SUMF2CO2PROC(200-461) [Golgilumen]SPMVKO11xCbxE-3D-F10(32-179)[Golgi lumen]11xCbxE-3D-F10(32-179)[endoplasmicreticulum lumen]active ARSCO23D-F9(29-461)CO2VKHQARSfactor VIIO2H2OCO2H2SGGCXPROS1(25-676)pro-factor XSUMF2 [endoplasmicreticulum lumen]12xCbxE-3D-F9(29-461)factor XEIF5A(Dhp)factor X heavy chain[endoplasmicreticulum lumen]H2OCO2protein C11xCbxE-PROS112xCbxE-3D-F9(47-461)3D-PROC(33-197)[endoplasmicreticulum lumen]PROZ(24-40)H+O2SUMF1 [endoplasmicreticulum lumen]DHPS [cytosol]H+3D-F10(32-179)[endoplasmicreticulum lumen]pro-prothrombin(factor II)VKHQO28xCbxE-3D-PROC(33-197)[Golgi lumen]8xCbxE-3D-PROC(43-197)[Golgi lumen]pro-protein CVKOPROC(200-461) [Golgilumen]11xCbxE-3D-F10(41-179)[Golgi lumen]pro-factor X,uncarboxylatedfactor X heavy chain[Golgi lumen]H2ONAD+H2OPROS1(25-41)GAS6(31-38)prothrombin (factorII) propeptide1,3-diaminopropanefactor X heavy chain[Golgi lumen]VKORC1CO2H2ONADH13xCbxE-PROZDOHH [cytosol]11xCbxE-GAS6(31-691)11xCbxE-PROS1(25-676)PROC(200-461)[endoplasmicreticulum lumen]DOHH:Fe++PROC(200-461)[endoplasmicreticulum lumen]factor IX propeptideVKOVKOfactor VIIpropeptideFe2+ [cytosol]FURIN10xCbxE-F2(25-622)10xCbxE-F7(21-466)10xCbxE-F2(44-622)VKHQpro-factor XSUMF111xCbxE-GAS6(39-691)11xCbxE-GAS6(31-691)O213xCbxE-PROZ(24-400)pro-factor VIIPROZ(24-400)EIF5A(Hyp)VKHQEIF5AVKO8xCbxE-3D-PROC(33-197)[endoplasmicreticulum lumen]GAS6(31-691)O2pro-protein C,uncarboxylated12xCbxE-3D-F9(29-461)factor X heavy chain[endoplasmicreticulum lumen]deoxyhypusinesynthase tetramer11xCbxE-PROS1(25-676)NADHPROC(33-42)13xCbxE-PROZ(24-400)10xCbxE-F2(25-622)CO2factor X light chainpropeptidepro-factor VIIVKHQNAD+SUMF2CO225, 269, 17, 3313215, 229, 17, 3319, 20, 2325, 2619, 2015, 2219, 20, 233, 239, 17, 33323, 231115, 22323, 2325, 2619, 2019, 20, 233, 2319, 20, 2319, 2015, 2225, 2632111132119, 17, 339, 17, 3319, 2015, 2219, 20, 233, 2325, 26


Description

After translation, many newly formed proteins undergo further covalent modifications that alter their functional properties and that are essentially irreversible under physiological conditions in the body. These modifications include the vitamin K-dependent attachment of carboxyl groups to glutamate residues and the conversion of lysine residues to hypusine.Original Pathway at Reactome: http://www.reactome.org/PathwayBrowser/#DB=gk_current&FOCUS_SPECIES_ID=48887&FOCUS_PATHWAY_ID=163841

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Bibliography

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  69. Preusser-Kunze A, Mariappan M, Schmidt B, Gande SL, Mutenda K, Wenzel D, von Figura K, Dierks T.; ''Molecular characterization of the human Calpha-formylglycine-generating enzyme.''; PubMed Europe PMC Scholia
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  75. Tie JK, Jin DY, Stafford DW.; ''Conserved loop cysteines of vitamin K epoxide reductase complex subunit 1-like 1 (VKORC1L1) are involved in its active site regeneration.''; PubMed Europe PMC Scholia
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History

View all...
CompareRevisionActionTimeUserComment
114840view16:34, 25 January 2021ReactomeTeamReactome version 75
113286view11:35, 2 November 2020ReactomeTeamReactome version 74
112497view15:45, 9 October 2020ReactomeTeamReactome version 73
101409view11:29, 1 November 2018ReactomeTeamreactome version 66
100947view21:05, 31 October 2018ReactomeTeamreactome version 65
100484view19:39, 31 October 2018ReactomeTeamreactome version 64
100029view16:23, 31 October 2018ReactomeTeamreactome version 63
99582view14:55, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99204view12:43, 31 October 2018ReactomeTeamreactome version 62
94013view13:51, 16 August 2017ReactomeTeamreactome version 61
93632view11:29, 9 August 2017ReactomeTeamreactome version 61
87451view13:58, 22 July 2016MkutmonOntology Term : 'peptide and protein metabolic process' added !
86744view09:25, 11 July 2016ReactomeTeamreactome version 56
83388view11:04, 18 November 2015ReactomeTeamVersion54
81771view10:19, 26 August 2015ReactomeTeamVersion53
77021view08:31, 17 July 2014ReactomeTeamFixed remaining interactions
76726view12:09, 16 July 2014ReactomeTeamFixed remaining interactions
76052view10:11, 11 June 2014ReactomeTeamRe-fixing comment source
75761view11:26, 10 June 2014ReactomeTeamReactome 48 Update
75111view14:06, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74861view18:44, 2 May 2014KhanspersChanged comment source for description
74758view08:50, 30 April 2014ReactomeTeamNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
1,3-diaminopropaneMetaboliteCHEBI:15725 (ChEBI)
10xCbxE-F2(25-622)ProteinP00734 (Uniprot-TrEMBL)
10xCbxE-F2(44-622)ProteinP00734 (Uniprot-TrEMBL)
10xCbxE-F7(21-466)ProteinP08709 (Uniprot-TrEMBL)
11xCbxE-3D-F10(32-179) [Golgi lumen]ProteinP00742 (Uniprot-TrEMBL)
11xCbxE-3D-F10(32-179)

[endoplasmic

reticulum lumen]
ProteinP00742 (Uniprot-TrEMBL)
11xCbxE-3D-F10(41-179) [Golgi lumen]ProteinP00742 (Uniprot-TrEMBL)
11xCbxE-GAS6(31-691)ProteinQ14393 (Uniprot-TrEMBL)
11xCbxE-GAS6(39-691)ProteinQ14393 (Uniprot-TrEMBL)
11xCbxE-PROS1(25-676)ProteinP07225 (Uniprot-TrEMBL)
11xCbxE-PROS1ProteinP07225 (Uniprot-TrEMBL)
12xCbxE-3D-F9(29-461)ProteinP00740 (Uniprot-TrEMBL)
12xCbxE-3D-F9(47-461)ProteinP00740 (Uniprot-TrEMBL)
13xCbxE-PROZ(24-400)ProteinP22891 (Uniprot-TrEMBL)
13xCbxE-PROZProteinP22891 (Uniprot-TrEMBL)
3D-F10(32-179)

[endoplasmic

reticulum lumen]
ProteinP00742 (Uniprot-TrEMBL)
3D-F9(29-461)ProteinP00740 (Uniprot-TrEMBL)
3D-PROC(33-197)

[endoplasmic

reticulum lumen]
ProteinP04070 (Uniprot-TrEMBL)
8xCbxE-3D-PROC(33-197) [Golgi lumen]ProteinP04070 (Uniprot-TrEMBL)
8xCbxE-3D-PROC(33-197)

[endoplasmic

reticulum lumen]
ProteinP04070 (Uniprot-TrEMBL)
8xCbxE-3D-PROC(43-197) [Golgi lumen]ProteinP04070 (Uniprot-TrEMBL)
ARSREACT_124132 (Reactome)
CO2MetaboliteCHEBI:16526 (ChEBI)
DHPS [cytosol]ProteinP49366 (Uniprot-TrEMBL)
DOHH [cytosol]ProteinQ9BU89 (Uniprot-TrEMBL)
DOHH:Fe++ComplexREACT_12867 (Reactome)
EIF5A(Dhp)ProteinREACT_161036 (Reactome)
EIF5A(Hyp)ProteinREACT_12952 (Reactome)
EIF5AProteinREACT_160986 (Reactome)
FURINProteinP09958 (Uniprot-TrEMBL)
Fe2+ [cytosol]MetaboliteCHEBI:18248 (ChEBI)
GAS6(31-38)ProteinQ14393 (Uniprot-TrEMBL)
GAS6(31-691)ProteinQ14393 (Uniprot-TrEMBL)
GGCXProteinP38435 (Uniprot-TrEMBL)
H+MetaboliteCHEBI:15378 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
H2SMetaboliteCHEBI:16136 (ChEBI)
NAD+MetaboliteCHEBI:15846 (ChEBI)
NADHMetaboliteCHEBI:16908 (ChEBI)
O2MetaboliteCHEBI:15379 (ChEBI)
PROC(200-461)

[endoplasmic

reticulum lumen]
ProteinP04070 (Uniprot-TrEMBL)
PROC(200-461) [Golgi lumen]ProteinP04070 (Uniprot-TrEMBL)
PROC(33-42)ProteinP04070 (Uniprot-TrEMBL)
PROS1(25-41)ProteinP07225 (Uniprot-TrEMBL)
PROS1(25-676)ProteinP07225 (Uniprot-TrEMBL)
PROZ(24-40)ProteinP22891 (Uniprot-TrEMBL)
PROZ(24-400)ProteinP22891 (Uniprot-TrEMBL)
SPMMetaboliteCHEBI:16610 (ChEBI)
SUMF1 [endoplasmic reticulum lumen]ProteinQ8NBK3 (Uniprot-TrEMBL)
SUMF1:SUMF2ComplexREACT_123836 (Reactome)
SUMF1ProteinQ8NBK3 (Uniprot-TrEMBL)
SUMF2 [endoplasmic reticulum lumen]ProteinQ8NBJ7 (Uniprot-TrEMBL)
SUMF2ProteinQ8NBJ7 (Uniprot-TrEMBL)
VKHQMetaboliteCHEBI:28433 (ChEBI)
VKOMetaboliteCHEBI:28371 (ChEBI)
VKORC1ProteinQ9BQB6 (Uniprot-TrEMBL)
active ARSREACT_122851 (Reactome)
deoxyhypusine synthase tetramerComplexREACT_13167 (Reactome)
factor IX propeptideProteinP00740 (Uniprot-TrEMBL)
factor VII propeptideProteinP08709 (Uniprot-TrEMBL)
factor VIIProteinP08709 (Uniprot-TrEMBL)
factor X heavy chain [Golgi lumen]ProteinP00742 (Uniprot-TrEMBL)
factor X heavy chain

[endoplasmic

reticulum lumen]
ProteinP00742 (Uniprot-TrEMBL)
factor X light chain propeptideProteinP00742 (Uniprot-TrEMBL)
factor XComplexREACT_3453 (Reactome)
pro-factor VIIProteinP08709 (Uniprot-TrEMBL)
pro-factor X, uncarboxylatedComplexREACT_4182 (Reactome)
pro-factor XComplexREACT_5090 (Reactome)
pro-factor XComplexREACT_5343 (Reactome)
pro-protein C, uncarboxylatedComplexREACT_3534 (Reactome)
pro-protein CComplexREACT_5288 (Reactome)
pro-protein CComplexREACT_5866 (Reactome)
pro-prothrombin (factor II)ProteinP00734 (Uniprot-TrEMBL)
protein CComplexREACT_5618 (Reactome)
prothrombin (factor II) propeptideProteinP00734 (Uniprot-TrEMBL)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
1,3-diaminopropaneArrowREACT_12551 (Reactome)
1,3-diaminopropaneREACT_12481 (Reactome)
10xCbxE-F2(25-622)ArrowREACT_46 (Reactome)
10xCbxE-F2(25-622)ArrowREACT_903 (Reactome)
10xCbxE-F2(25-622)REACT_1065 (Reactome)
10xCbxE-F2(25-622)REACT_46 (Reactome)
10xCbxE-F2(44-622)ArrowREACT_1065 (Reactome)
10xCbxE-F7(21-466)ArrowREACT_1969 (Reactome)
10xCbxE-F7(21-466)REACT_1743 (Reactome)
11xCbxE-GAS6(31-691)ArrowREACT_1290 (Reactome)
11xCbxE-GAS6(31-691)ArrowREACT_1 (Reactome)
11xCbxE-GAS6(31-691)REACT_1117 (Reactome)
11xCbxE-GAS6(31-691)REACT_1 (Reactome)
11xCbxE-GAS6(39-691)ArrowREACT_1117 (Reactome)
11xCbxE-PROS1(25-676)ArrowREACT_1307 (Reactome)
11xCbxE-PROS1(25-676)ArrowREACT_199 (Reactome)
11xCbxE-PROS1(25-676)REACT_1860 (Reactome)
11xCbxE-PROS1(25-676)REACT_199 (Reactome)
11xCbxE-PROS1ArrowREACT_1860 (Reactome)
12xCbxE-3D-F9(29-461)ArrowREACT_2045 (Reactome)
12xCbxE-3D-F9(29-461)ArrowREACT_618 (Reactome)
12xCbxE-3D-F9(29-461)REACT_420 (Reactome)
12xCbxE-3D-F9(29-461)REACT_618 (Reactome)
12xCbxE-3D-F9(47-461)ArrowREACT_420 (Reactome)
13xCbxE-PROZ(24-400)ArrowREACT_124 (Reactome)
13xCbxE-PROZ(24-400)ArrowREACT_1864 (Reactome)
13xCbxE-PROZ(24-400)REACT_1535 (Reactome)
13xCbxE-PROZ(24-400)REACT_1864 (Reactome)
13xCbxE-PROZArrowREACT_1535 (Reactome)
3D-F9(29-461)REACT_2045 (Reactome)
ARSREACT_121125 (Reactome)
CO2REACT_124 (Reactome)
CO2REACT_1290 (Reactome)
CO2REACT_1307 (Reactome)
CO2REACT_1565 (Reactome)
CO2REACT_1969 (Reactome)
CO2REACT_2045 (Reactome)
CO2REACT_903 (Reactome)
CO2REACT_913 (Reactome)
DOHH:Fe++mim-catalysisREACT_12479 (Reactome)
EIF5A(Dhp)ArrowREACT_12551 (Reactome)
EIF5A(Dhp)REACT_12479 (Reactome)
EIF5A(Dhp)REACT_12481 (Reactome)
EIF5A(Hyp)ArrowREACT_12479 (Reactome)
EIF5AArrowREACT_12481 (Reactome)
EIF5AREACT_12551 (Reactome)
FURINmim-catalysisREACT_1065 (Reactome)
FURINmim-catalysisREACT_1117 (Reactome)
FURINmim-catalysisREACT_1423 (Reactome)
FURINmim-catalysisREACT_1535 (Reactome)
FURINmim-catalysisREACT_1644 (Reactome)
FURINmim-catalysisREACT_1860 (Reactome)
FURINmim-catalysisREACT_2146 (Reactome)
FURINmim-catalysisREACT_420 (Reactome)
GAS6(31-38)ArrowREACT_1117 (Reactome)
GAS6(31-691)REACT_1290 (Reactome)
GGCXmim-catalysisREACT_124 (Reactome)
GGCXmim-catalysisREACT_1290 (Reactome)
GGCXmim-catalysisREACT_1307 (Reactome)
GGCXmim-catalysisREACT_1565 (Reactome)
GGCXmim-catalysisREACT_1969 (Reactome)
GGCXmim-catalysisREACT_2045 (Reactome)
GGCXmim-catalysisREACT_903 (Reactome)
GGCXmim-catalysisREACT_913 (Reactome)
H+ArrowREACT_12551 (Reactome)
H+REACT_12481 (Reactome)
H+REACT_950 (Reactome)
H2OArrowREACT_124 (Reactome)
H2OArrowREACT_1290 (Reactome)
H2OArrowREACT_1307 (Reactome)
H2OArrowREACT_1565 (Reactome)
H2OArrowREACT_1969 (Reactome)
H2OArrowREACT_2045 (Reactome)
H2OArrowREACT_903 (Reactome)
H2OArrowREACT_913 (Reactome)
H2SArrowREACT_121125 (Reactome)
NAD+ArrowREACT_12481 (Reactome)
NAD+REACT_121125 (Reactome)
NAD+REACT_12551 (Reactome)
NADHArrowREACT_121125 (Reactome)
NADHArrowREACT_12551 (Reactome)
O2REACT_12479 (Reactome)
O2REACT_124 (Reactome)
O2REACT_1290 (Reactome)
O2REACT_1307 (Reactome)
O2REACT_1565 (Reactome)
O2REACT_1969 (Reactome)
O2REACT_2045 (Reactome)
O2REACT_903 (Reactome)
O2REACT_913 (Reactome)
PROC(33-42)ArrowREACT_2146 (Reactome)
PROS1(25-41)ArrowREACT_1860 (Reactome)
PROS1(25-676)REACT_1307 (Reactome)
PROZ(24-40)ArrowREACT_1535 (Reactome)
PROZ(24-400)REACT_124 (Reactome)
REACT_1065 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-prothrombin (factor II)' is present. At the end of this reaction, 1 molecule of 'prothrombin (factor II) propeptide', and 1 molecule of 'prothrombin (factor II)' are present.

This reaction takes place in the 'Golgi membrane' and is mediated by the 'furin activity' of 'furin'.

REACT_1103 (Reactome) In this reaction, 1 molecule of 'pro-protein C' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_1117 (Reactome) The details of the gamma-carboxylation of GAS6 have not been determined directly, but are inferred from those worked out for protein S (Manfioletti et al. 1993).
REACT_121125 (Reactome) The sulfatase-modifying factor 1 (SUMF1, also called C-alpha-formylglycine-generating enzyme, FGE) (Preusser-Kunze et al. 2005, Cosma et al. 2003, Landgrebe et al. 2003) oxidises the critical cysteine residue in arylsulfatases to an active site 3-oxoalanine residue thus confering sulfatase activity (Roeser et al. 2006). Defects in SUMF1 cause multiple sulfatase deficiency (MSD) (MIM:272200), an impairment of arylsulfatase activity due to defective post-translational modification of the cysteine residue (Cosma et al. 2003, Dierks et al, 2003). This post-translational modification is thought to be highly conserved in eukaryotes (Selmer et al. 1996, von Figura et al. 1998). SUMF1 is active as either a monomer or a homodimer. A monomer is described in this reaction.
REACT_121184 (Reactome) Sulfatase-modifying factor 2 (SUMF2, also called C-alpha-formylglycine-generating enzyme 2, pFGE) is the paralogue of SUMF1. While SUMF1 can modify a critical residue on arylsulfatases to confer activity to them, SUMF2 lacks this ability (Mariappan et al. 2005) and instead, SUMF2 can inhibit the action of SUMF1 by dimerising with it (Zito et al. 2005). SUMF2 can interact with sulfatases with and without SUMF1 (Zito et al. 2005).
REACT_12479 (Reactome) Cytosolic deoxyhypusine hydroxylase catalyzes the irreversible conversion of peptidyl-deoxyhypusine to peptidyl-hypusine. The only known substrate for this enzyme is the modified lysine at residue 50 of eIF5A (Kang et al. 2007; Kim et al. 2006).
REACT_12481 (Reactome) The reaction of EIF5A, spermidine, and NAD+ to form EIF5A(Dhp), 1,3-diaminopropane, and NADH + H+ is reversible in vitro. Under physiological conditions, the reverse reaction is probably minimized by the rapid, irreversible conversion of FIF5A(Dhp) to EIF5A(Hyp).
REACT_124 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-protein Z, uncarboxylated', 13 molecules of 'Oxygen', 13 molecules of 'vitamin K hydroquinone', and 13 molecules of 'CO2' are present. At the end of this reaction, 1 molecule of 'pro-protein Z', 13 molecules of 'H2O', and 13 molecules of 'vitamin K epoxide' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_12551 (Reactome) Cytosolic deoxyhypusine synthase catalyzes the reaction of EIF5A protein, spermidine, and NAD+ to convert lysine-50 of EIF5A to deoxyhypusine, generating 1,3-diaminopropane and NADH + H+ in the process (Park 2006). Although the reaction is reversible, the reverse reaction is probably minimized under physiological conditions by the rapid, irreversible conversion of EIF5A(Dhp) to EIF5A(Hyp).
REACT_1290 (Reactome) The details of the gamma-carboxylation of GAS6 have not been determined directly, but are inferred from those worked out for protein S (Manfioletti et al. 1993).
REACT_1307 (Reactome) At the beginning of this reaction, 11 molecules of 'Oxygen', 11 molecules of 'vitamin K hydroquinone', 11 molecules of 'CO2', and 1 molecule of 'pro-protein S, uncarboxylated' are present. At the end of this reaction, 1 molecule of 'pro-protein S', 11 molecules of 'H2O', and 11 molecules of 'vitamin K epoxide' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_1423 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-factor VII' is present. At the end of this reaction, 1 molecule of 'factor VII', and 1 molecule of 'factor VII propeptide' are present.

This reaction takes place in the 'Golgi membrane' and is mediated by the 'furin activity' of 'furin'.

REACT_1535 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-protein Z' is present. At the end of this reaction, 1 molecule of 'protein Z propeptide', and 1 molecule of 'protein Z' are present.

This reaction takes place in the 'Golgi lumen' and is mediated by the 'furin activity' of 'furin'.

REACT_1565 (Reactome) At the beginning of this reaction, 11 molecules of 'Oxygen', 1 molecule of 'pro-factor X, uncarboxylated', 11 molecules of 'vitamin K hydroquinone', and 11 molecules of 'CO2' are present. At the end of this reaction, 1 molecule of 'pro-factor X', 11 molecules of 'H2O', and 11 molecules of 'vitamin K epoxide' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_1644 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-factor X' is present. At the end of this reaction, 1 molecule of 'factor X light chain propeptide', and 1 molecule of 'factor X' are present.

This reaction takes place in the 'Golgi membrane' and is mediated by the 'furin activity' of 'furin'.

REACT_1743 (Reactome) In this reaction, 1 molecule of 'pro-factor VII' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_1860 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-protein S' is present. At the end of this reaction, 1 molecule of 'protein S propeptide', and 1 molecule of 'protein S' are present.

This reaction takes place in the 'Golgi lumen' and is mediated by the 'furin activity' of 'furin'.

REACT_1864 (Reactome) In this reaction, 1 molecule of 'pro-protein Z' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_1969 (Reactome) At the beginning of this reaction, 10 molecules of 'Oxygen', 10 molecules of 'vitamin K hydroquinone', 10 molecules of 'CO2', and 1 molecule of 'pro-factor VII, uncarboxylated' are present. At the end of this reaction, 1 molecule of 'pro-factor VII', 10 molecules of 'H2O', and 10 molecules of 'vitamin K epoxide' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_199 (Reactome) In this reaction, 1 molecule of 'pro-protein S' is translocated from endoplasmic reticulum membrane to Golgi membrane.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_1 (Reactome) In this reaction, 1 molecule of 'pro-GAS6' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_2045 (Reactome) At the beginning of this reaction, 12 molecules of 'Oxygen', 12 molecules of 'vitamin K hydroquinone', 12 molecules of 'CO2', and 1 molecule of 'pro-factor IX, uncarboxylated' are present. At the end of this reaction, 12 molecules of 'H2O', 12 molecules of 'vitamin K epoxide', and 1 molecule of 'pro-factor IX' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_2146 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-protein C' is present. At the end of this reaction, 1 molecule of 'protein C', and 1 molecule of 'protein C light chain propeptide' are present.

This reaction takes place in the 'Golgi lumen' and is mediated by the 'furin activity' of 'furin'.

REACT_420 (Reactome) At the beginning of this reaction, 1 molecule of 'pro-factor IX' is present. At the end of this reaction, 1 molecule of 'factor IX', and 1 molecule of 'factor IX propeptide' are present.

This reaction takes place in the 'Golgi membrane' and is mediated by the 'furin activity' of 'furin'.

REACT_46 (Reactome) In this reaction, 1 molecule of 'pro-prothrombin (factor II)' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_618 (Reactome) In this reaction, 1 molecule of 'pro-factor IX' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

REACT_903 (Reactome) At the beginning of this reaction, 10 molecules of 'Oxygen', 10 molecules of 'vitamin K hydroquinone', 10 molecules of 'CO2', and 1 molecule of 'pro-prothrombin (factor II), uncarboxylated' are present. At the end of this reaction, 1 molecule of 'pro-prothrombin (factor II)', 10 molecules of 'H2O', and 10 molecules of 'vitamin K epoxide' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_913 (Reactome) At the beginning of this reaction, 8 molecules of 'Oxygen', 8 molecules of 'vitamin K hydroquinone', 8 molecules of 'CO2', and 1 molecule of 'pro-protein C, uncarboxylated' are present. At the end of this reaction, 8 molecules of 'H2O', 8 molecules of 'vitamin K epoxide', and 1 molecule of 'pro-protein C' are present.

This reaction takes place in the 'endoplasmic reticulum membrane' and is mediated by the 'gamma-glutamyl carboxylase activity' of 'vitamin K-dependent gamma-carboxylase'.

REACT_950 (Reactome) The regeneration of reduced vitamin K (vitamin K hydroquinone) from vitamin K epoxide is catalyzed by vitamin K epoxide reductase (VKORC1) (Sadler 2004). This enzyme is the target of the anticoagulant drug warfarin. Two important features of this reaction remain unclear. First, dithiothreitol functions efficiently as a reductant in vitro (Wallin and Martin 1985), but the in vivo reductant remains unknown. Second, while people homozygous for mutations in VKORC1 protein lack epoxide reductase activity (Rost et al. 2004) and cultured insect cells transfected with the cloned human VKORC1 gene express vitamin K epoxide reductase activity (Li et al. 2004), the possibility that the active form of the enzyme is a complex with other proteins cannot be formally excluded.
REACT_968 (Reactome) In this reaction, 1 molecule of 'pro-factor X' is translocated from endoplasmic reticulum lumen to Golgi lumen.

This reaction takes place in the 'ER to Golgi transport vesicle'.

SPMArrowREACT_12481 (Reactome)
SPMREACT_12551 (Reactome)
SUMF1:SUMF2ArrowREACT_121184 (Reactome)
SUMF1REACT_121184 (Reactome)
SUMF1mim-catalysisREACT_121125 (Reactome)
SUMF2REACT_121184 (Reactome)
SUMF2TBarREACT_121125 (Reactome)
VKHQArrowREACT_950 (Reactome)
VKHQREACT_124 (Reactome)
VKHQREACT_1290 (Reactome)
VKHQREACT_1307 (Reactome)
VKHQREACT_1565 (Reactome)
VKHQREACT_1969 (Reactome)
VKHQREACT_2045 (Reactome)
VKHQREACT_903 (Reactome)
VKHQREACT_913 (Reactome)
VKOArrowREACT_124 (Reactome)
VKOArrowREACT_1290 (Reactome)
VKOArrowREACT_1307 (Reactome)
VKOArrowREACT_1565 (Reactome)
VKOArrowREACT_1969 (Reactome)
VKOArrowREACT_2045 (Reactome)
VKOArrowREACT_903 (Reactome)
VKOArrowREACT_913 (Reactome)
VKORC1mim-catalysisREACT_950 (Reactome)
VKOREACT_950 (Reactome)
active ARSArrowREACT_121125 (Reactome)
deoxyhypusine synthase tetramermim-catalysisREACT_12481 (Reactome)
deoxyhypusine synthase tetramermim-catalysisREACT_12551 (Reactome)
factor IX propeptideArrowREACT_420 (Reactome)
factor VII propeptideArrowREACT_1423 (Reactome)
factor VIIArrowREACT_1423 (Reactome)
factor X light chain propeptideArrowREACT_1644 (Reactome)
factor XArrowREACT_1644 (Reactome)
pro-factor VIIArrowREACT_1743 (Reactome)
pro-factor VIIREACT_1423 (Reactome)
pro-factor VIIREACT_1969 (Reactome)
pro-factor X, uncarboxylatedREACT_1565 (Reactome)
pro-factor XArrowREACT_1565 (Reactome)
pro-factor XArrowREACT_968 (Reactome)
pro-factor XREACT_1644 (Reactome)
pro-factor XREACT_968 (Reactome)
pro-protein C, uncarboxylatedREACT_913 (Reactome)
pro-protein CArrowREACT_1103 (Reactome)
pro-protein CArrowREACT_913 (Reactome)
pro-protein CREACT_1103 (Reactome)
pro-protein CREACT_2146 (Reactome)
pro-prothrombin (factor II)REACT_903 (Reactome)
protein CArrowREACT_2146 (Reactome)
prothrombin (factor II) propeptideArrowREACT_1065 (Reactome)
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