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Literature summary for 2.8.1.12 extracted from

  • Leimkuhler, S.; Freuer, A.; Araujo, J.A.; Rajagopalan, K.V.; Mendel, R.R.
    Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency (2003), J. Biol. Chem., 278, 26127-26134.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
MOCS2A and MOCS2B genotyping, recombinant expression of human subunits MOCS2A and MOCS2B in Escherichia coli strain BL21(DE3), functional complementation of Escherichia coli moaD and moaE mutants. In vitro translation and mutagenesis experiments of MOCS2A and MOCS2B Homo sapiens

Protein Variants

Protein Variants Comment Organism
E168K naturally occuring substitution in MOCS2B, the E168K mutation, identified in a severely affected patient, attenuates binding of precursor Z, the MPT synthase tetramer is readily formed in mixtures of MOCS2B-E168K with equimolar amounts of MOCS2A Homo sapiens
additional information a deletion of Ala150 is introduced into MOCS2B, attempts to purify MOCS2B-A150DELTA fails because the protein forms inclusion bodies upon expression in the Escherichia coli cells. Construction of a MOCS2B variant lacking the first 43 amino acid residues, the DELTA1-43 variant of MOCS2B is unstable because the majority of the protein was rapidly degraded during or after purification Homo sapiens
V7F naturally occuring substitution in MOCS2A, identified in a patient with an unusual mild form of the disease, the mutation weakens the interaction between MOCS2A and MOCS2B, the MOCS2A-V7F variant does not form a complex with MOCS2B in either its carboxylated or thiocarboxylated form, and the mutant MPT synthase shows 90% reduced activity compared to the wild-type enzyme Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
9800
-
2 * 9800, subunit MOCS2A, + 2 * 20800, subunit MCS2B, SDS-PAGE Homo sapiens
20800
-
2 * 9800, subunit MOCS2A, + 2 * 20800, subunit MCS2B, SDS-PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
cyclic pyranopterin phosphate + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH2-C(O)SH + H2O Homo sapiens
-
molybdopterin + 2 molybdopterin-synthase sulfur-carrier protein
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens O96007
-
-

Purification (Commentary)

Purification (Comment) Organism
recombinant human subunits MOCS2A and MOCS2B from Escherichia coli strain BL21(DE3) by ammonium sulfate fractionation and gel filtration. The separately purified subunits readily assemble into a functional MPT synthase tetramer Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
cyclic pyranopterin phosphate + 2 [molybdopterin-synthase sulfur-carrier protein]-Gly-NH-CH2-C(O)SH + H2O
-
Homo sapiens molybdopterin + 2 molybdopterin-synthase sulfur-carrier protein
-
?

Subunits

Subunits Comment Organism
heterotetramer 2 * 9800, subunit MOCS2A, + 2 * 20800, subunit MCS2B, SDS-PAGE Homo sapiens
More human MPT synthase contains the subunits MOCS2A and MOCS2B Homo sapiens

Synonyms

Synonyms Comment Organism
MPT synthase
-
Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
22
-
assay at room temperature Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.2
-
assay at Homo sapiens

General Information

General Information Comment Organism
malfunction characterization of mutants identified in group B patients of molybdenum cofactor deficiency, molecular mechanism leading to human molybdenum cofactor deficiency, overview Homo sapiens
additional information the rate of conversion of precursor Z to MPT by the human enzyme is slower than that of the eubacterial homologue from Escherichia coli Homo sapiens