title: beta-N-acetylhexosaminidases in the spleen of a patient with hairy-cell leukaemia.
abstract: The spleen from a patient with hairy-cell leukaemia had beta-N-acetylhexosaminidase activity that could be resolved into three isoenzymes by chromatography on phenyl boronate agarose. Two of these were the major forms, A and B, found in normal tissues but, in addition, there was an 'extra' form that accounted for 15% of total activity. The 'extra' form hydrolysed the synthetic substrate 4-methylumbelliferyl-beta-N-acetylglucosamine 6-sulphate, indicating the presence of alpha-subunits. It was more acidic than A, was less heat-stable and showed no generation of B on denaturation under a variety of conditions. These findings and the immunoblot (Western blotting) analysis demonstrate that the 'extra' form is entirely composed of alpha-subunits, and most closely resembles S, the residual activity in Sandhoff's disease.
authors: Dipartimento di Medicina Sperimentale e Scienze Biochimiche, Università di Perugia, Italy.
authors: C
authors: C
authors: Emiliani
authors: R
authors: R
authors: Sciarra
authors: A
authors: A
authors: Orlacchio
authors: J L
authors: JL
authors: Stirling
mesh_terms: D015153:Blotting, Western; D002845:Chromatography; D004355:Drug Stability; D006358:Hot Temperature; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007525:Isoelectric Focusing; D007527:Isoenzymes / Q000037:antagonists & inhibitors / Q000302:isolation & purification* / Q000378:metabolism; D007700:Kinetics; D007943:Leukemia, Hairy Cell / Q000201:enzymology*; D013154:Spleen / Q000201:enzymology*; D001619:beta-N-Acetylhexosaminidases / Q000037:antagonists & inhibitors / Q000302:isolation & purification* / Q000378:metabolism
chemical_list: D007527:Isoenzymes; D001619:beta-N-Acetylhexosaminidases