Retarded bone formation in GM1-gangliosidosis: a study of the infantile form and comparison with two canine models
Retarded bone formation in GM1-gangliosidosis: a study of the infantile form and comparison with two canine models
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GM1-神经节苷脂沉积症的骨形成延迟：婴儿形式的研究以及与两种犬模型的比较
作者：
J. Alroy;K. Knowles;S. Schelling;E. Kaye;A. Rosenberg
The development of skeletal lesions in two canine models of GM1-gangliosidosis, English springer spaniels and Portuguese water dogs, has been studied and compared to osseous abnormalities in a child with the infantile form of the disease. In the canine models, skeletal dysplasia was progressive. Lesions were noted at 2 months of age and characterized by retarded endochondral ossification and osteoporosis. Older puppies had focal cartilage necrosis within lumbar vertebral epiphyses. At the cellular level, lesions were characterized by chondrocytic hypertrohy and lysosomal accumulation of storage compounds. Our studies illustrate that the skeletal lesions in both canine models are similar to those in a child with GM1-gangliosidosis. Furthermore, we proposed that the abnormal storage of partially degraded compounds in affected chondrocytes might explain, at least in part, the retarded bone formation noted in patients with GM1-gangliosidosis.
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DOI：
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发表时间：
1980
期刊：
The American journal of pathology
影响因子：
--
作者：
Haskins,ME;Aguirre,GD;Jezyk,PF;Patterson,DF
通讯作者：
Patterson,DF
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DOI：
--
发表时间：
1992
期刊：
The American journal of pathology
影响因子：
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作者：
Alroy,J;Orgad,U;DeGasperi,R;Richard,R;Warren,CD;Knowles,K;Thalhammer,JG;Raghavan,SS
通讯作者：
Raghavan,SS
猫粘多糖贮积症模型的病理学Ⅰ。
DOI：
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发表时间：
1983
期刊：
The American journal of pathology
影响因子：
--
作者：
Haskins,ME;Aguirre,GD;Jezyk,PF;Desnick,RJ;Patterson,DF
通讯作者：
Patterson,DF
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DOI：
--
发表时间：
1990
期刊：
The American journal of pathology
影响因子：
--
作者：
Vogler,C;Birkenmeier,EH;Sly,WS;Levy,B;Pegors,C;Kyle,JW;Beamer,WG
通讯作者：
Beamer,WG