Abstract
Considering the formation of excessive membrane structures which may induce lowering of physiological activities or subsequent death of affected cells, the elucidation of structure and its forming process of these cytoplasmic inclusion bodies (CIB) will presu-mably contribute to clarify the mechanism of lipid accumulation suppressing the cellular functions.
The present report documents the analysis of CIB protein obtained from four patients with Gaucher's disease (adult form) by use of the semi-micro polyacrylamide disc gradient gel, which had been developed by Abe and Ebato.
The CIB of Gaucher's disease did not show myelin-like spiral structure as seen in those of Tay-Sachs and Niemman-Pick disease, but an elongated tubular structure. None of the twisted structure repoted by Lee et al. was clearly demonstrated.
In all four samples, coomassie blue stain revealed five major bands and five faint bands of high-molecular weight protein. The molecular weights for five major bands were estimated to be 2.7×104, 4.2×104, 5.8×104, 6.4×104 and 8.6×104, respectively, and the maximum molecular weight for the highest-molecular protein bands was 2.1×105 .
It is notewarthy to point out that the CIB electropholetic pattern showed an protein band of the same mobility as the tubulin, major part of constitutional elements of micro tubular system, suggesting that tubulin may play an important role for the formation of the CIB in metabolic disorder of lipid.
It is interesting that four cases of Gaucher's disease presented identical CIB electrophoretic pattern. This may suggest that the membranous structures are formed in organs and tissues under certain uniformly regulated conditions. There are two possible basic biochemical nodes of CIB formation, that is, protein incorporated to lipid base, or vice versa.
The reproducibility and sharpness of the semi-micro polyacrylamid disc gradient gel were proved.
