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4FLC

Structural and Biochemical Characterization of Human Adenylosuccinate Lyase (ADSL) and the R303C ADSL Deficiency Associated Mutation

Functional Information from GO Data
ChainGOidnamespacecontents
A0001666biological_processresponse to hypoxia
A0003824molecular_functioncatalytic activity
A0004018molecular_functionN6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) activity
A0005829cellular_componentcytosol
A0006164biological_processpurine nucleotide biosynthetic process
A0006167biological_processAMP biosynthetic process
A0006177biological_processGMP biosynthetic process
A0006189biological_process'de novo' IMP biosynthetic process
A0007584biological_processresponse to nutrient
A0009060biological_processaerobic respiration
A0009152biological_processpurine ribonucleotide biosynthetic process
A0014850biological_processresponse to muscle activity
A0016829molecular_functionlyase activity
A0032991cellular_componentprotein-containing complex
A0042594biological_processresponse to starvation
A0042802molecular_functionidentical protein binding
A0044208biological_process'de novo' AMP biosynthetic process
A0044209biological_processAMP salvage
A0070626molecular_function(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido) succinate lyase (fumarate-forming) activity
A0097294biological_process'de novo' XMP biosynthetic process
B0001666biological_processresponse to hypoxia
B0003824molecular_functioncatalytic activity
B0004018molecular_functionN6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) activity
B0005829cellular_componentcytosol
B0006164biological_processpurine nucleotide biosynthetic process
B0006167biological_processAMP biosynthetic process
B0006177biological_processGMP biosynthetic process
B0006189biological_process'de novo' IMP biosynthetic process
B0007584biological_processresponse to nutrient
B0009060biological_processaerobic respiration
B0009152biological_processpurine ribonucleotide biosynthetic process
B0014850biological_processresponse to muscle activity
B0016829molecular_functionlyase activity
B0032991cellular_componentprotein-containing complex
B0042594biological_processresponse to starvation
B0042802molecular_functionidentical protein binding
B0044208biological_process'de novo' AMP biosynthetic process
B0044209biological_processAMP salvage
B0070626molecular_function(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido) succinate lyase (fumarate-forming) activity
B0097294biological_process'de novo' XMP biosynthetic process
C0001666biological_processresponse to hypoxia
C0003824molecular_functioncatalytic activity
C0004018molecular_functionN6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) activity
C0005829cellular_componentcytosol
C0006164biological_processpurine nucleotide biosynthetic process
C0006167biological_processAMP biosynthetic process
C0006177biological_processGMP biosynthetic process
C0006189biological_process'de novo' IMP biosynthetic process
C0007584biological_processresponse to nutrient
C0009060biological_processaerobic respiration
C0009152biological_processpurine ribonucleotide biosynthetic process
C0014850biological_processresponse to muscle activity
C0016829molecular_functionlyase activity
C0032991cellular_componentprotein-containing complex
C0042594biological_processresponse to starvation
C0042802molecular_functionidentical protein binding
C0044208biological_process'de novo' AMP biosynthetic process
C0044209biological_processAMP salvage
C0070626molecular_function(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido) succinate lyase (fumarate-forming) activity
C0097294biological_process'de novo' XMP biosynthetic process
D0001666biological_processresponse to hypoxia
D0003824molecular_functioncatalytic activity
D0004018molecular_functionN6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) activity
D0005829cellular_componentcytosol
D0006164biological_processpurine nucleotide biosynthetic process
D0006167biological_processAMP biosynthetic process
D0006177biological_processGMP biosynthetic process
D0006189biological_process'de novo' IMP biosynthetic process
D0007584biological_processresponse to nutrient
D0009060biological_processaerobic respiration
D0009152biological_processpurine ribonucleotide biosynthetic process
D0014850biological_processresponse to muscle activity
D0016829molecular_functionlyase activity
D0032991cellular_componentprotein-containing complex
D0042594biological_processresponse to starvation
D0042802molecular_functionidentical protein binding
D0044208biological_process'de novo' AMP biosynthetic process
D0044209biological_processAMP salvage
D0070626molecular_function(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido) succinate lyase (fumarate-forming) activity
D0097294biological_process'de novo' XMP biosynthetic process
Functional Information from PROSITE/UniProt
site_idPS00163
Number of Residues10
DetailsFUMARATE_LYASES Fumarate lyases signature. GSsaMpYKrN
ChainResidueDetails
AGLY288-ASN297

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues4
DetailsActive site: {"description":"Proton donor/acceptor","evidences":[{"source":"PubMed","id":"22812634","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI2
Number of Residues8
DetailsBinding site: {}
ChainResidueDetails

site_idSWS_FT_FI3
Number of Residues28
DetailsBinding site: {"description":"in other chain"}
ChainResidueDetails

site_idSWS_FT_FI4
Number of Residues7
DetailsModified residue: {"description":"N6-acetyllysine","evidences":[{"source":"PubMed","id":"19608861","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

site_idSWS_FT_FI5
Number of Residues8
DetailsCross-link: {"description":"Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO1)","evidences":[{"source":"PubMed","id":"25114211","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails

244349

PDB entries from 2025-11-05

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