Biochimica et biophysica acta · 1990
beta-N-acetylhexosaminidases in the spleen of a patient with hairy-cell leukaemia.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Emiliani C, Sciarra R, Orlacchio A, Stirling J L
Аннотация
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.