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Proteins/UNIPROT:Q8N142

ADSS1

Protein·UNIPROT:Q8N142Adenylosuccinate synthetase isozyme 1

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Component of the purine nucleotide cycle (PNC), which interconverts IMP and AMP to regulate the nucleotide levels in various tissues, and which contributes to glycolysis and ammoniagenesis. Catalyzes the first committed step in the biosynthesis of AMP from IMP..

Function from UniProtKB (Q8N142), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

1 row

myopathy, distal, 5 has a germline basis in ADSS1DiseaseGenCC HGNC:20093|MONDO:0014877Inferred only

Statements

43 statements

43 statements · 7 reference databases · Subject: ADSS1 · Sources: Bgee, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, UniProt

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MAEA covaries with ADSS1ProteinHetionet hetionet:covaries:10296-122622Inferred only
ADSS1 covaries with OTUB1ProteinHetionet hetionet:covaries:122622-55611Inferred only
SIAH1 covaries with ADSS1ProteinHetionet hetionet:covaries:122622-6477Inferred only
ADSS1 is expressed in aortaAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in apex of heartAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in biceps brachiiAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in body of tongueAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in cardiac atriumAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in cardiac muscle of right atriumAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in cardiac ventricleAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in deltoidAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in gastrocnemiusAnatomyBgee ENSG00000185100Inferred only

Show the remaining 31

ADSS1 is expressed in heartAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in heart left ventricleAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in heart right ventricleAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in hindlimb stylopod muscleAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in left testisAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in left ventricle myocardiumAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in muscle tissueAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in myocardiumAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in myocardiumAnatomyHuman Protein Atlas ENSG00000185100Inferred only
ADSS1 is expressed in popliteal arteryAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in quadriceps femorisAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in right atrium auricular regionAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in Skeletal muscle tissueAnatomyHuman Protein Atlas ENSG00000185100Inferred only
ADSS1 is expressed in Skeletal muscle tissueAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in skeletal muscle tissue of biceps brachiiAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in skeletal muscle tissue of rectus abdominisAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in tibialis anteriorAnatomyBgee ENSG00000185100Inferred only
ADSS1 is expressed in vastus lateralisAnatomyBgee ENSG00000185100Inferred only
AZIN1 interacts with ADSS1ProteinHetionet hetionet:interacts:122622-51582Inferred only
LYAR interacts with ADSS1ProteinHetionet hetionet:interacts:122622-55646Inferred only
YWHAZ interacts with ADSS1ProteinHetionet hetionet:interacts:122622-7534Inferred only
ADSS1 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
ADSS1 participates in Metabolism of nucleotidesPathwayReactome R-HSA-15869Inferred only
ADSS1 participates in Nucleotide biosynthesisPathwayReactome R-HSA-8956320Inferred only
ADSS1 participates in Purine ribonucleoside monophosphate biosynthesisPathwayReactome R-HSA-73817Inferred only
ADSS1 requires the cofactor magnesium(2+)CompoundUniProt Q8N142Inferred only
CHEBI:17202 is a substrate of ADSS1CompoundHuman-GEM MAR04042Inferred only
IMP is a substrate of ADSS1CompoundRhea RHEA:15753Inferred only
L-aspartate is a substrate of ADSS1CompoundHuman-GEM MAR04042Inferred only
L-aspartate is a substrate of ADSS1CompoundRhea RHEA:15753Inferred only
N(6)-(1,2-dicarboxyethyl)-AMP is a substrate of ADSS1CompoundRhea RHEA:15753Inferred only

Reactions

2 reactions

L-aspartate+GTP+CHEBI:17202N(6)-(1,2-dicarboxyethyl)-AMP+GDP+2proton+phosphate(3-)
Catalysed by ADSS2 or ADSS1
Reaction pageLeft to rightBalancedEC 6.3.4.4cytosol
Human-GEM MAR04042CC BY 4.0Reference data
IMP+L-aspartate+GTP

Other names

8 names

Resolves to: ADSS1

Also called: Adenylosuccinate synthetase-like 1Adenylosuccinate synthetase, basic isozymeAdenylosuccinate synthetase, muscle isozymeAdSS 1ADSSL1AMPSase 1IMP--aspartate ligase 1M-type adenylosuccinate synthetase

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N(6)-(1,2-dicarboxyethyl)-AMP

+hydrogenphosphate

Catalysed by ADSS2 or ADSS1

Reaction pageDirection not assertedBalanced

Rhea RHEA:15753CC BY 4.0Reference data