Hepatic ultrastructure in the Hurler syndrome.
Hepatic ultrastructure in the Hurler syndrome.
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Hurler 综合征中的肝脏超微结构。
DOI：
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发表时间：
1966
影响因子：
6
通讯作者：
A. Lorincz
中科院分区：
文献类型：
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作者：
W. P. Callahan;A. Lorincz
The heritable disorder of man known as the Hurler syndrome (lipochondrodystrophy, gargoylism, dysostosis multiplex) has in the past decade advanced from the status of a rare medical curiosity to a clinical problem of considerable research interest. It is a biochemical disorder of connective tissue metabolism, which may be transmitted either as a sex-linked recessive affecting only males, or as a simple autosomal recessive affecting both sexes.1'2 This disorder is characterized by excessive excretion in urine and excessive accumulation in liver and other tissues of the acid mucopolysaccharides, chondroitin sulfuric acid-B (/3-heparin) and heparin monosulfuric acid (heparitin).3-7 The chemical nature of these carbohydrate polymers, which are usually complexed with proteins in their natural state, has rendered difficult their precise identification and localization by available histochemical techniques.8 Studies of Hurler liver based on histochemical and morphologic observations at the level of light microscopy have not clearly resolved the question as to whether these mucopolysaccharide-protein substances exist primarily intracellularly or extracellularly.9'13 Our earlier studies of liver ultrastructure in the Hurler syndrome clearly demonstrated the intracytoplasmic vacuolar accumulation of these glycoprotein substances. Morphologically these vacuoles appeared to be comparable to similar glycoprotein accumulations in cells of the poison glands of reptiles.14 Orizaga, Haust, Bryans and Frank,15 also observed the intracellular accumulation of this material thus confirming our initial findings.'4 They further stated, however, that all other cellular organelles, including lysosomes, appeared unaltered except for the mitochondria which were increased in density and reduced in number. Our observations on mitochondria and lysosomes1" were not in agreement with these statements. Subsequent work by Van Hoof and Hers 17 on liver ultrastructure in Hurler's syndrome confirmed our findings. In an attempt to elucidate the mechanism of mucopolysaccharide storage in the liver and to determine whether specific cellular organelles
