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Literature summary extracted from

  • Kress, B.C.; Hirani, S.; Freeze, H.H.; Little, L.; Miller, A.L.
    Mucolipidosis III beta-N-acetyl-D-hexosaminidase A. Purification and properties (1982), Biochem. J., 207, 421-428.
    View publication on PubMedView publication on EuropePMC

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.2.1.52 extracellular in mucolipidosis patients Homo sapiens
-
-
3.2.1.52 lysosome in healthy humans Homo sapiens 5764
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.52 26000
-
 1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE Homo sapiens
3.2.1.52 29000
-
 1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme Homo sapiens
3.2.1.52 29500
-
 1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme Homo sapiens
3.2.1.52 54000
-
 1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE Homo sapiens
3.2.1.52 55000
-
 1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme Homo sapiens
3.2.1.52 55600
-
 1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme Homo sapiens
3.2.1.52 58000
-
 1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme Homo sapiens
3.2.1.52 59600
-
 1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme Homo sapiens
3.2.1.52 68000
-
 1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.2.1.52 N-acetyl-beta-D-hexosaminides + H2O Homo sapiens
-
 N-acetyl-D-hexosamines
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.52 Homo sapiens
-
 healthy persons and mucolipidosis patients
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.2.1.52 glycoprotein mucolipidosis enzyme shows decreased carbohydrate composition compared to normal enzyme Homo sapiens
3.2.1.52 glycoprotein carbohydrate modification Homo sapiens
3.2.1.52 glycoprotein increased amount of galactose present on mucolipidosis enzyme compared to normal enzyme Homo sapiens
3.2.1.52 glycoprotein galactosylation Homo sapiens

Purification (Commentary)

EC Number Purification (Comment) Organism
3.2.1.52 to homogeneity, normal enzyme 64863fold, I-cell enzyme 20140fold Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.52 placenta
-
 Homo sapiens
-
3.2.1.52 urine
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.2.1.52 77.84
-
 substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside, normal enzyme Homo sapiens
3.2.1.52 90.63
-
 substrate: 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside, I-cell enzyme Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.52 4-methylumbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside + H2O
-
 Homo sapiens 4-methylumbelliferone + 2-acetamido-2-deoxy-beta-D-glucopyranoside
-
 ?
3.2.1.52 N-acetyl-beta-D-hexosaminides + H2O
-
 Homo sapiens N-acetyl-D-hexosamines
-
 ?

Subunits

EC Number Subunits Comment Organism
3.2.1.52 dimer 1 * 54000 + 1 * 26000, placental enzyme, SDS-PAGE Homo sapiens
3.2.1.52 tetramer 1 * 68000 + 1 * 59600 + 1 * 55600 + 1 * 29500, SDS-PAGE, mucolipidosis enzyme Homo sapiens
3.2.1.52 trimer 1 * 58000 + 1 * 55000 + 1 * 29000, SDS-PAGE, normal enzyme Homo sapiens