Glyoxylate metabolism and glycine degradation (Homo sapiens)

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362911, 16, 25, 4411, 16, 25, 44142, 37, 4331, 383912, 3026, 41, 421, 273434, 354, 13, 21, 2434187, 486, 9, 28, 40, 53820, 23, 46, 47, 503, 22, 5410, 175, 3319mitochondrial matrixcytosolperoxisomal matrixNAD+DHsperoxisomalglyoxylate carrierPXMP2 DDO CO2H+GlyD-AspGlyAdoHcyHOGA1 tetramerBCKDHB lipo-K-DHTKD1 GRHPRglycolateFAD PYRGCSHLIPT2H2ODHLL GNMT NH3L-AspDLD NDUFAB1 PDHX glyoxylateGCSH GOT2 dimeroctanoyl group L-AlaPDHB PXLP-GOT2 LIPT1PDHA1 HAO1 tetramerHAO1 DLD OXPYRNADP+GCSH:SAMDLLFAD PDHA2 HPROGLDC SAMDLL NADH5,10-methylene-THFLIPAM lipoyl-K107-GCSH4-OH-L-GluFAD PDHA2 HPROoctanoyl-K107-GCSHDDO:FADH2O1-pyrroline-3-hydroxy-5-carboxylatePDHB H+NH4+glycolateHOGFAD SGCSH:DHLLDAO PDHA1 BCKDHA PXLP THFlipo-K44-DBT H2O2glyoxylatePXMP2 trimerPDHX PRODH2 dimerFMN NADHBCKDHB NDUFAB1PRODH2 LIPAM 4-OH-L-GlusemialdehydeLipo-K110-DLST AdoMetGLDC dimer:2xPXLPDHTKD1 H2O25dAdeO2H2OOAFAD TDP TDP DBT AMTOAAGCSH O2LIAS:2(4Fe-4S)ALDH4A1 dimerH+GNMT tetramer4Fe-4S cluster PXLP-AGXT2 DLST DLD dimer:2xFADDLAT DLD DAO dimerOGDH FAD L-AlaglyoxylateBCKDHA HPRO carrierAGXT dimerALDH4A1 lipoylated DHsoctanoyl:NDUFAB1SARCH2OAGXT2 tetramerL-Metlipo-K132,K259-DLAT AdoMetPXLP-AGXT NAD+LIAS NADPHHOGA1 OGDH O2plasma membraneglyoxylate carrierGly6, 285251485015, 52514532, 3815, 5255249515232, 3855244050


Glyoxylate is generated in the course of glycine and hydroxyproline catabolism and can be converted to oxalate. In humans, this process takes place in the liver. Defects in two enzymes of glyoxylate metabolism, alanine:glyoxylate aminotransferase (AGXT) and glycerate dehydrogenase/glyoxylate reductase (GRHPR), are associated with pathogenic overproduction of oxalate (Danpure 2005). The reactions that interconvert glycine, glycolate, and glyoxylate and convert glyoxylate to oxalate have been characterized in molecular detail in humans. A reaction sequence for the conversion of hydroxyproline to glyoxylate has been inferred from studies of partially purified extracts of rat and bovine liver but the enzymes involved in the corresponding human reactions have not been identified. View original pathway at:Reactome.


Pathway is converted from Reactome ID: 389661
Reactome version: 66
Reactome Author 
Reactome Author: D'Eustachio, Peter

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101238view11:13, 1 November 2018ReactomeTeamreactome version 66
100777view20:40, 31 October 2018ReactomeTeamreactome version 65
100320view19:17, 31 October 2018ReactomeTeamreactome version 64
99865view16:00, 31 October 2018ReactomeTeamreactome version 63
99422view14:36, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99105view12:39, 31 October 2018ReactomeTeamreactome version 62
93997view13:50, 16 August 2017ReactomeTeamreactome version 61
93606view11:28, 9 August 2017ReactomeTeamreactome version 61
87455view14:02, 22 July 2016MkutmonOntology Term : 'glyoxylate and dicarboxylate metabolic pathway' added !
86713view09:24, 11 July 2016ReactomeTeamreactome version 56
83452view12:26, 18 November 2015ReactomeTeamNew pathway

External references


View all...
NameTypeDatabase referenceComment
1-pyrroline-3-hydroxy-5-carboxylateMetaboliteCHEBI:27391 (ChEBI)
4-OH-L-Glu semialdehydeMetaboliteCHEBI:62637 (ChEBI)
4-OH-L-GluMetaboliteCHEBI:32812 (ChEBI)
4Fe-4S cluster MetaboliteCHEBI:49883 (ChEBI)
5,10-methylene-THFMetaboliteCHEBI:12071 (ChEBI)
5dAdeMetaboliteCHEBI:17319 (ChEBI)
AGXT dimerComplexR-HSA-389671 (Reactome)
AGXT2 tetramerComplexR-HSA-904854 (Reactome)
ALDH4A1 ProteinP30038 (Uniprot-TrEMBL)
ALDH4A1 dimerComplexR-HSA-70674 (Reactome)
AMTProteinP48728 (Uniprot-TrEMBL)
AdoHcyMetaboliteCHEBI:16680 (ChEBI)
AdoMetMetaboliteCHEBI:15414 (ChEBI)
BCKDHA ProteinP12694 (Uniprot-TrEMBL)
BCKDHB ProteinP21953 (Uniprot-TrEMBL)
CO2MetaboliteCHEBI:16526 (ChEBI)
D-AspMetaboliteCHEBI:17364 (ChEBI)
DAO ProteinP14920 (Uniprot-TrEMBL)
DAO dimerComplexR-HSA-389849 (Reactome)
DBT ProteinP11182 (Uniprot-TrEMBL)
DDO ProteinQ99489 (Uniprot-TrEMBL)
DDO:FADComplexR-HSA-6810064 (Reactome)
DHLL MetaboliteCHEBI:50746 (ChEBI)
DHTKD1 ProteinQ96HY7 (Uniprot-TrEMBL)
DHsComplexR-HSA-6792579 (Reactome)
DLAT ProteinP10515 (Uniprot-TrEMBL)
DLD ProteinP09622 (Uniprot-TrEMBL)
DLD dimer:2xFADComplexR-HSA-5694020 (Reactome)
DLST ProteinP36957 (Uniprot-TrEMBL)
FAD MetaboliteCHEBI:16238 (ChEBI)
FMN MetaboliteCHEBI:17621 (ChEBI)
GCSH ProteinP23434 (Uniprot-TrEMBL)
GCSH:DHLLComplexR-HSA-5693982 (Reactome)
GCSH:SAMDLLComplexR-HSA-5693969 (Reactome)
GCSHProteinP23434 (Uniprot-TrEMBL)
GLDC ProteinP23378 (Uniprot-TrEMBL)
GLDC dimer:2xPXLPComplexR-HSA-5693954 (Reactome)
GNMT ProteinQ14749 (Uniprot-TrEMBL)
GNMT tetramerComplexR-HSA-6798322 (Reactome)
GOT2 dimerComplexR-HSA-70594 (Reactome)
GRHPRProteinQ9UBQ7 (Uniprot-TrEMBL)
GlyMetaboliteCHEBI:57305 (ChEBI)
H+MetaboliteCHEBI:15378 (ChEBI)
H2O2MetaboliteCHEBI:16240 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
HAO1 ProteinQ9UJM8 (Uniprot-TrEMBL)
HAO1 tetramerComplexR-HSA-389845 (Reactome)
HOGA1 ProteinQ86XE5 (Uniprot-TrEMBL)
HOGA1 tetramerComplexR-HSA-6784409 (Reactome)
HOGMetaboliteCHEBI:17742 (ChEBI)
HPRO carrierR-HSA-8953316 (Reactome)
HPROMetaboliteCHEBI:18240 (ChEBI)
L-AlaMetaboliteCHEBI:57972 (ChEBI)
L-AspMetaboliteCHEBI:29991 (ChEBI)
L-MetMetaboliteCHEBI:57844 (ChEBI)
LIAS ProteinO43766 (Uniprot-TrEMBL)
LIAS:2(4Fe-4S)ComplexR-HSA-6793623 (Reactome)
LIPAM MetaboliteCHEBI:17460 (ChEBI)
LIPT1ProteinQ9Y234 (Uniprot-TrEMBL)
LIPT2ProteinA6NK58 (Uniprot-TrEMBL)
Lipo-K110-DLST ProteinP36957 (Uniprot-TrEMBL)
NAD+MetaboliteCHEBI:15846 (ChEBI)
NADHMetaboliteCHEBI:16908 (ChEBI)
NADP+MetaboliteCHEBI:18009 (ChEBI)
NADPHMetaboliteCHEBI:16474 (ChEBI)
NDUFAB1 ProteinO14561 (Uniprot-TrEMBL)
NDUFAB1ProteinO14561 (Uniprot-TrEMBL)
NH3MetaboliteCHEBI:16134 (ChEBI)
NH4+MetaboliteCHEBI:28938 (ChEBI)
O2MetaboliteCHEBI:15379 (ChEBI)
OAAMetaboliteCHEBI:30744 (ChEBI)
OAMetaboliteCHEBI:30744 (ChEBI)
OGDH ProteinQ02218 (Uniprot-TrEMBL)
OXMetaboliteCHEBI:16995 (ChEBI)
PDHA1 ProteinP08559 (Uniprot-TrEMBL)
PDHA2 ProteinP29803 (Uniprot-TrEMBL)
PDHB ProteinP11177 (Uniprot-TrEMBL)
PDHX ProteinO00330 (Uniprot-TrEMBL)
PRODH2 ProteinQ9UF12 (Uniprot-TrEMBL)
PRODH2 dimerComplexR-HSA-6784240 (Reactome)
PXLP MetaboliteCHEBI:18405 (ChEBI)
PXLP-AGXT ProteinP21549 (Uniprot-TrEMBL)
PXLP-AGXT2 ProteinQ9BYV1 (Uniprot-TrEMBL)
PXLP-GOT2 ProteinP00505 (Uniprot-TrEMBL)
PXMP2 ProteinQ9NR77 (Uniprot-TrEMBL)
PXMP2 trimerComplexR-HSA-8953434 (Reactome)
PYRMetaboliteCHEBI:32816 (ChEBI)
SAMDLL MetaboliteCHEBI:14949 (ChEBI)
SARCMetaboliteCHEBI:15611 (ChEBI)
SMetaboliteCHEBI:26833 (ChEBI)
TDP MetaboliteCHEBI:18290 (ChEBI)
THFMetaboliteCHEBI:15635 (ChEBI)
glycolateMetaboliteCHEBI:29805 (ChEBI)
glyoxylateMetaboliteCHEBI:16891 (ChEBI)
lipo-K-DHTKD1 ProteinQ96HY7 (Uniprot-TrEMBL)
lipo-K132,K259-DLAT ProteinP10515 (Uniprot-TrEMBL)
lipo-K44-DBT ProteinP11182 (Uniprot-TrEMBL)
lipoyl-K107-GCSHProteinP23434 (Uniprot-TrEMBL)
lipoylated DHsComplexR-HSA-6792580 (Reactome)
octanoyl group MetaboliteCHEBI:25650 (ChEBI)
octanoyl-K107-GCSHProteinP23434 (Uniprot-TrEMBL)
octanoyl:NDUFAB1ComplexR-HSA-6793596 (Reactome)
peroxisomal glyoxylate carrierR-HSA-8953410 (Reactome)
plasma membrane glyoxylate carrierR-HSA-8953409 (Reactome)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
1-pyrroline-3-hydroxy-5-carboxylateArrowR-HSA-6784224 (Reactome)
1-pyrroline-3-hydroxy-5-carboxylateR-HSA-6784402 (Reactome)
4-OH-L-Glu semialdehydeArrowR-HSA-6784402 (Reactome)
4-OH-L-Glu semialdehydeR-HSA-6784399 (Reactome)
4-OH-L-GluArrowR-HSA-6784399 (Reactome)
4-OH-L-GluR-HSA-6784393 (Reactome)
5,10-methylene-THFArrowR-HSA-5693977 (Reactome)
5dAdeArrowR-HSA-6793591 (Reactome)
AGXT dimermim-catalysisR-HSA-389684 (Reactome)
AGXT2 tetramermim-catalysisR-HSA-904864 (Reactome)
ALDH4A1 dimermim-catalysisR-HSA-6784399 (Reactome)
AMTmim-catalysisR-HSA-5693977 (Reactome)
AdoHcyArrowR-HSA-6798317 (Reactome)
AdoMetR-HSA-6793591 (Reactome)
AdoMetR-HSA-6798317 (Reactome)
CO2ArrowR-HSA-5693967 (Reactome)
D-AspR-HSA-6810076 (Reactome)
DAO dimermim-catalysisR-HSA-389821 (Reactome)
DDO:FADmim-catalysisR-HSA-6810076 (Reactome)
DHsR-HSA-6792572 (Reactome)
DLD dimer:2xFADmim-catalysisR-HSA-5694018 (Reactome)
GCSH:DHLLArrowR-HSA-5693977 (Reactome)
GCSH:DHLLR-HSA-5694018 (Reactome)
GCSH:SAMDLLArrowR-HSA-5693967 (Reactome)
GCSH:SAMDLLR-HSA-5693977 (Reactome)
GCSHArrowR-HSA-6792572 (Reactome)
GCSHR-HSA-6793590 (Reactome)
GLDC dimer:2xPXLPmim-catalysisR-HSA-5693967 (Reactome)
GNMT tetramermim-catalysisR-HSA-6798317 (Reactome)
GOT2 dimermim-catalysisR-HSA-6784393 (Reactome)
GRHPRmim-catalysisR-HSA-389826 (Reactome)
GlyArrowR-HSA-389684 (Reactome)
GlyArrowR-HSA-904864 (Reactome)
GlyR-HSA-389821 (Reactome)
GlyR-HSA-5693967 (Reactome)
GlyR-HSA-6798317 (Reactome)
H+ArrowR-HSA-5694018 (Reactome)
H+ArrowR-HSA-6784399 (Reactome)
H+R-HSA-389826 (Reactome)
H2O2ArrowR-HSA-389821 (Reactome)
H2O2ArrowR-HSA-389842 (Reactome)
H2O2ArrowR-HSA-389862 (Reactome)
H2O2ArrowR-HSA-6810076 (Reactome)
H2OR-HSA-389821 (Reactome)
H2OR-HSA-389862 (Reactome)
H2OR-HSA-6784402 (Reactome)
H2OR-HSA-6810076 (Reactome)
HAO1 tetramermim-catalysisR-HSA-389842 (Reactome)
HAO1 tetramermim-catalysisR-HSA-389862 (Reactome)
HOGA1 tetramermim-catalysisR-HSA-6784423 (Reactome)
HOGArrowR-HSA-6784393 (Reactome)
HOGR-HSA-6784423 (Reactome)
HPRO carriermim-catalysisR-HSA-6784213 (Reactome)
HPROArrowR-HSA-6784213 (Reactome)
HPROR-HSA-6784213 (Reactome)
HPROR-HSA-6784224 (Reactome)
L-AlaR-HSA-389684 (Reactome)
L-AlaR-HSA-904864 (Reactome)
L-AspArrowR-HSA-6784393 (Reactome)
L-MetArrowR-HSA-6793591 (Reactome)
LIAS:2(4Fe-4S)mim-catalysisR-HSA-6793591 (Reactome)
LIPT1mim-catalysisR-HSA-6792572 (Reactome)
LIPT2mim-catalysisR-HSA-6793590 (Reactome)
NAD+R-HSA-5694018 (Reactome)
NAD+R-HSA-6784399 (Reactome)
NADHArrowR-HSA-5694018 (Reactome)
NADHArrowR-HSA-6784399 (Reactome)
NADP+ArrowR-HSA-389826 (Reactome)
NADPHR-HSA-389826 (Reactome)
NDUFAB1ArrowR-HSA-6793590 (Reactome)
NH3ArrowR-HSA-5693977 (Reactome)
NH4+ArrowR-HSA-389821 (Reactome)
NH4+ArrowR-HSA-6810076 (Reactome)
O2R-HSA-389821 (Reactome)
O2R-HSA-389842 (Reactome)
O2R-HSA-389862 (Reactome)
O2R-HSA-6810076 (Reactome)
OAAR-HSA-6784393 (Reactome)
OAArrowR-HSA-6810076 (Reactome)
OXArrowR-HSA-389862 (Reactome)
PRODH2 dimermim-catalysisR-HSA-6784224 (Reactome)
PXMP2 trimermim-catalysisR-HSA-8953430 (Reactome)
PYRArrowR-HSA-389684 (Reactome)
PYRArrowR-HSA-6784423 (Reactome)
PYRArrowR-HSA-904864 (Reactome)
R-HSA-389684 (Reactome) Alanine-glyoxylate transaminase (AGXT) catalyzes the irreversible reaction of glyoxylate and alanine to form glycine and pyruvate (Danpure and Jennings 1988). The active form of the enzyme is a homodimer (Zhang et al. 2003) with one molecule of pyridoxal phosphate bound to each subunit (Coulter-Mackie et al. 2005). Mutations in this enzyme are associated with primary hyperoxaluria type I. Mutant alleles encode both catalytically inactive proteins and active ones that are mis-localized to mitochondria (Purdue et al. 1990; Takada et al. 1990).
R-HSA-389821 (Reactome) Peroxisomal D-amino-acid oxidase catalyzes the reaction of glycine, water, and O2 to form glyoxylate, H2O2, and NH4+. The active form of the enzyme is a homodimer and has FAD as a cofactor (Kawazoe et al. 2006; Molla et al. 2006).
R-HSA-389826 (Reactome) Peroxisomal GRHPR catalyzes the reaction of glyoxylate and NADPH + H+ to form glycolate and NADP+. The active form of the enzyme is a monomer (Rumsby and Cregeen 1999); mutations in it are associated with primary hyperoxaluria type II (Cramer et al. 1999).
R-HSA-389842 (Reactome) Peroxisomal hydroxyacid oxidase 1 catalyzes the reaction of glycolate and O2 to form glyoxylate and H2O2. The active form of the enzyme is associated with FMN and is a tetramer (Jones et al. 2000; Murray et al. 2008; Vignaud et al. 2007; Williams et al. 2000).
R-HSA-389862 (Reactome) Peroxisomal hydroxyacid oxidase 1 catalyzes the reaction of glyoxylate to form oxalate. The active form of the enzyme is associated with FMN and is a tetramer (Jones et al. 2000; Murray et al. 2008; Vignaud et al. 2007; Williams et al. 2000).
R-HSA-5693967 (Reactome) The simplest amino acid, glycine, is catabolised by several different pathways. The major pathway is via the glycine cleavage system. In the first reaction, glycine (Gly) is decarboxylated to carbon dioxide (CO2) and aminomethyl group (NH2CH2) by mitochondrial glycine dehydrogenase (decarboxylating) (GLDC, P protein), a dimeric protein using pyridoxal 5-phosphate (PXPL) as cofactor per subunit (Kume et al. 1991). Mitochondrial glycine cleavage system H protein (GCSH) is used as a co-substrate in this reaction. GCSH uses lipoate as a cofactor which accepts the aminomethylgroup from glycine decarboxylation to form a S-aminomethyldihydrolipoylated protein (GCSH:SAMDLL) (Fujiwara et al. 1991, Fujiwara et al. 1991).
R-HSA-5693977 (Reactome) The major degradative pathway for the amino acid glycine is via the glycine cleavage system. In the second reaction in this system, the decarboxylated moiety from glycine decarboxylation attached to H protein (GCSH:SAMDLL) is further degraded by mitochondrial aminomethyltransferase (AMT, GCST, T protein) to ammonia (NH3) and GCSH with reduced lipoate. Tetrahydrofolate (THF) is required for this reaction and accepts the methyl group to form 5,10MTHF (Fujiwara et al. 1984).
R-HSA-5694018 (Reactome) The last step in the glycine cleavage system is the reoxidation of the reduced lipoate (dihydrolipoyl group) attached to the H protein (GCSH:DHLL) catalysed by the L protein (mitochondrial dihydrolipoyl dehydrogenase, DLD) (Harris et al. 1997, Ciszak et al. 2006).
R-HSA-6784213 (Reactome) Cytosolic hydroxyproline (HPRO) is transported into the mitochonrial matrix in a saturable process distinct from the one responsible for proline uptake. The carrier that mediates this process has not been identified, however (Atlante et al. 1996).
R-HSA-6784224 (Reactome) PRODH2 dimer dehydrogenates hydroxyproline (HPRO) to form 1-pyrroline-3-hydroxy-5-carboxylate (Adams & Goldstone 1960). The enzyme is associated with FAD and ubiquinone (not annotated here) is the likely electron acceptor (Summitt et al. 2015). The mitochondrial localization of the reaction is inferred from studies of HPRO catabolism in rat and bovine systems (Adams & Frank 1980), and the localization of PRODH2 to the inner mitochondrial membrane is inferred from that of the homologous mouse protein (Da Cruz et al. 2003).
R-HSA-6784393 (Reactome) GOT2 dimer transaminates 4-OH-L-glutamate (4-OH-L-Glu) and oxaloacetate (OA) to form 4-hydroxy-2-oxoglutarate (HOG) and L-Asp. The ability of human GOT2 to catalyze this reaction has been inferred from studies of its rat homologue (Maitra & Dekker 1964).
R-HSA-6784399 (Reactome) Mitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) catalyzes the reaction of 4-hydroxy-L-glutamate gamma-semialdehyde and NAD+ to form 4-hydroxyglutamate and NADH + H+. ALDH4A1 also catalyzes the corresponding reaction of proline catabolism, as shown in biochemical and structural studies (Adams & Goldstone 1960; Srivastava et al. 2012), and mutations in ALDH4A1 disrupt both catabolic processes in human patients (Valle et al. 1979).
R-HSA-6784402 (Reactome) The spontaneous hydrolysis of 1-pyrroline-3-hydroxy-5-carboxylate to form 4-OH-L-glutamate semialdehyde is inferred from the behavior of the analogous intermediate of proline catabolism (Moxley et al. 2011).
R-HSA-6784423 (Reactome) Mitochondrial HOGA1 aldol-cleaves 4-OH-2-oxoglutarate (HOG) to glyoxylate and pyruvate. The biochemical details of the enzyme are inferred from the properties of its well-studied rat homologue (Maitra & Dekker 1964). The mature protein lacks a 25-residue mitochondrial targeting sequence and forms a homotetramer (Riedel et al. 2011).
R-HSA-6784434 (Reactome) Cytosolic glyoxylate can enter the peroxisome but the carrier that mediates its entry has not been identified (Wanders et al. 2016).
R-HSA-6784436 (Reactome) Glyoxylate generated in the mitochondrion can enter the cytosol but the carrier that mediates its entry has not been identified (Wanders et al. 2016).
R-HSA-6792572 (Reactome) Lipoate is an essential cofactor for two enzymes from energy metabolism (alpha-ketoglutarate dehydrogenase and pyruvate dehydrogenase) and three from amino acid metabolism (branched-chain ketoacid dehydrogenase, 2-oxoadipate dehydrogenase, and the glycine cleavage system). Lipoate synthesis in mitochondria requires three steps. In the third step, mitochondrial lipoyltransferase 1 (LIPT1) catalyses the transfer of a lipoyl group from lipoyl-K107-GCSH to lysine residue(s) of lipoate-dependent enzymes (Fujiwara et al. 1999). Defects in LIPT1 reduce lipoylation of pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, causing a cofactor disorder of mitochondrial energy metabolism (Soreze et al. 2013, Tort et al. 2014).
R-HSA-6793590 (Reactome) Lipoate is an essential cofactor for five redox reactions; four in oxoacid dehydrogenases (active in energy metabolism and amino acid metabolism) and one in the glycine cleavage system (GCS). Lipoate synthesis in mitochondria requires three steps. In the first step, mitochondrial lipoyltransferase 2 (LIPT2) transfers an octanoyl group bound to an acyl-carrier protein (most likely NDUFAB1, acyl-carrier protein, ACP) to mitochondrial glycine cleavage system H protein (GCSH) at lysine 107. The human protein is thought to function in the same way as yeast LIP2 (Schonauer et al. 2009).
R-HSA-6793591 (Reactome) Lipoate is an essential cofactor for five redox reactions; four in oxoacid dehydrogenases (active in energy metabolism and amino acid metabolism) and one in the glycine cleavage system (GCS). Lipoate synthesis in mitochondria requires three steps. In the second step, mitochondrial lipoyl synthase (LIAS) mediates the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to glycine cleavage system H protein (GCSH), transforming the octanoyl moiety to a lipoyl moiety. LIAS requires two 4Fe-4S clusters as cofactor which act as the sulfur donors in the reaction (Morikawa et al. 2001). Defects in LIAS causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation (Mayr et al. 2011).
R-HSA-6798317 (Reactome) Cytosolic glycine N-methyltransferase (GNMT) catalyses the transfer of a methyl group from S-adenosylmethionine (AdoMet) to glycine (Gly) to form sarcosine (SARC, aka N-methylglycine) with the concomitant production of S-adenosylhomocysteine (AdoHcy) (Pakhomova et al. 2004).
R-HSA-6810076 (Reactome) Peroxisomal DDO (D-aspartate oxidase) catalyzes the oxidation of D-Asp (D-aspartate) to OA (oxaloacetate) with the formation of H2O2. The human enzyme is a monomer with an FAD cofactor (Katane et al. 2010, 2015; Setoyama & Miura 1997), as is its well-characterized bovine homolog (Negri et al. 1992). Its peroxisomal location is inferred from studies in cultured cells of fusion proteins containing the carboxyterminal peptide sequence of DDO (Amery et al. 1998).
R-HSA-8953430 (Reactome) Peroxisomal membrane protein 2 (PXMP2) homotrimer is inferred from the properties of its mouse homolog to form a channel in the peroxisomal membrane that allows the passage of glycolate and other molecules with molecular masses less than 200 Da between the cytosol and the peroxisomal matrix (Rokka et al. 2009; Wanders et al. 2016).
R-HSA-904864 (Reactome) Mitochondrial AGXT2 (alanine-glyoxylate transaminase 2) catalyzes the irreversible reaction of glyoxylate and alanine to form glycine and pyruvate (Rodionov et al. 2010). The active form of the enzyme is inferred to be a homotetramer from the properties of the homologous rat protein, which has been purified and characterized in vitro (Tamaki et al.990). Most conversion of glyoxylate to glycine in vivo appears to occur in the peroxisome, catalyzed by AGXT, and the physiological role of the AGXT2 reaction is unclear.
SARCArrowR-HSA-6798317 (Reactome)
SR-HSA-6793591 (Reactome)
THFR-HSA-5693977 (Reactome)
glycolateArrowR-HSA-389826 (Reactome)
glycolateArrowR-HSA-8953430 (Reactome)
glycolateR-HSA-389842 (Reactome)
glycolateR-HSA-8953430 (Reactome)
glyoxylateArrowR-HSA-389821 (Reactome)
glyoxylateArrowR-HSA-389842 (Reactome)
glyoxylateArrowR-HSA-6784423 (Reactome)
glyoxylateArrowR-HSA-6784434 (Reactome)
glyoxylateArrowR-HSA-6784436 (Reactome)
glyoxylateR-HSA-389684 (Reactome)
glyoxylateR-HSA-389826 (Reactome)
glyoxylateR-HSA-389862 (Reactome)
glyoxylateR-HSA-6784434 (Reactome)
glyoxylateR-HSA-6784436 (Reactome)
glyoxylateR-HSA-904864 (Reactome)
lipoyl-K107-GCSHArrowR-HSA-5694018 (Reactome)
lipoyl-K107-GCSHArrowR-HSA-6793591 (Reactome)
lipoyl-K107-GCSHR-HSA-5693967 (Reactome)
lipoyl-K107-GCSHR-HSA-6792572 (Reactome)
lipoylated DHsArrowR-HSA-6792572 (Reactome)
octanoyl-K107-GCSHArrowR-HSA-6793590 (Reactome)
octanoyl-K107-GCSHR-HSA-6793591 (Reactome)
octanoyl:NDUFAB1R-HSA-6793590 (Reactome)
peroxisomal glyoxylate carriermim-catalysisR-HSA-6784434 (Reactome)
plasma membrane glyoxylate carriermim-catalysisR-HSA-6784436 (Reactome)
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