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Literature summary extracted from

  • Kijas, A.W.; Harris, J.L.; Harris, J.M.; Lavin, M.F.
    Aprataxin forms a discrete branch in the HIT (histidine triad) superfamily of proteins with both DNA/RNA binding and nucleotide hydrolase activities (2006), J. Biol. Chem., 281, 13939-13948.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.6.1.71 expression in Saccharomyces cerevisiae Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.6.1.71 additional information removal of the N-terminal forkhead associated domain does not alter activity with substrates AMPNH2 and Ap4A Homo sapiens
3.6.1.71 V263G mutation identified in a AOA1 patient, mutant protein is unable to bind DNA Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.6.1.71 Duplex DNA significantly reduces catalytic catalytic activity on the model histidine triad nucleotide-binding protein substrate, AMPNH2, and the Fragile histidine triad protein substrate, Ap4A Homo sapiens
3.6.1.71 single-strand DNA reduces catalytic catalytic activity on the model histidine triad nucleotide-binding protein substrate, AMPNH2, and the Fragile histidine triad protein substrate, Ap4A Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.6.1.71 0.018
-
 P1,P4-bis(5'-adenosyl)tetraphosphate pH 7.0, 30°C Homo sapiens
3.6.1.71 0.837
-
 adenosine-5'-monophosphoramidate pH 7.0, 30°C Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
3.6.1.71 Homo sapiens Q7Z2E3
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.6.1.71 adenosine-5'-monophosphoramidate + H2O
-
 Homo sapiens AMP + NH3
-
 ?
3.6.1.71 ATP + H2O
-
 Homo sapiens AMP + diphosphate
-
 ?
3.6.1.71 additional information aprataxin hydrolyzes with similar efficiency the model histidine triad nucleotide-binding protein substrate : adenosine-5'-monophosphoramidate, AMPNH2, and the Fragile histidine triad protein substrate, Ap4A. No substrate: dATP Homo sapiens ?
-
 ?
3.6.1.71 P1,P4-bis(5'-adenosyl)tetraphosphate + H2O
-
 Homo sapiens AMP + ATP
-
 ?

Turnover Number [1/s]

EC Number Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
3.6.1.71 0.0115
-
 P1,P4-bis(5'-adenosyl)tetraphosphate pH 7.0, 30°C Homo sapiens
3.6.1.71 0.0195
-
 adenosine-5'-monophosphoramidate pH 7.0, 30°C Homo sapiens

General Information

EC Number General Information Comment Organism
3.6.1.71 physiological function aprataxin has dual DNA binding and nucleotide hydrolase activities. The protein binds to double-stranded DNA with high affinity but is also capable of binding double-stranded RNA and single-strand DNA, with increased affinity for hairpin structures. The DNA binding is not dependent on zinc Homo sapiens