beta-N-acetylhexosaminidases in the spleen of a patient with hairy-cell leukaemia |
| |
Authors: | C Emiliani R Sciarra A Orlacchio J L Stirling |
| |
Institution: | Dipartimento di Medicina Sperimentale e Scienze Biochimiche, Università di Perugia, Italy. |
| |
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. |
| |
Keywords: | |
|
|