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Literature summary for 3.2.1.52 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

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

Molecular Weight [Da]

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

Natural Substrates/ Products (Substrates)

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

Organism

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

Posttranslational Modification

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

Purification (Commentary)

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

Source Tissue

Source Tissue Comment Organism Textmining
placenta
-
Homo sapiens
-
urine
-
Homo sapiens
-

Specific Activity [micromol/min/mg]

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

Substrates and Products (Substrate)

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

Subunits

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