Characterization of osteopenia in feline mucopolysaccharidosis VI and evaluation of bone marrow transplantation therapy.
Characterization of osteopenia in feline mucopolysaccharidosis VI and evaluation of bone marrow transplantation therapy.
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猫粘多糖贮积症 VI 骨质减少的特征和骨髓移植治疗的评估。
DOI：
10.1016/8756-3282(93)90165-7
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发表时间：
1993
期刊：
影响因子：
4.1
通讯作者：
Gasper,PW
中科院分区：
文献类型：
--
作者：
Norrdin,RW;Moffat,KS;Thrall,MA;Gasper,PW
Mucopolysaccharidosis VI (MPS VI) is an inherited lysozymal storage disease in which a deficiency of aryl sulphatase 6 leads to accumulation of the glycosaminoglycan (GAG) dermatan sulphate. The condition is seen in man (Maroteaux-Lamy syndrome) and a feline model in Siamese cats (Haskins et al., 1980). The skeletal system changes include facial dysmorphism, stunting, and various abnormalities of bone formation resulting in deforming arthropathy, kyphosis and scoliosis (dysostosis multiplex). In the cat, there is also significant osteopenia. Histologically, there is cytoplasmic vacuolation and apparent enlargement of mesenchmal cells including fibroblasts, chondroblasts and osteoblasts.Bone marrow transplantation has been used on a limited basis to treat various mucopolysaccharidoses including MPS VI, in humans (Neufeld and Muenzer, 1989) and cats (Gasper et al., 1984). These studies indicate that skeletal changes may only be halted in adults but partly prevented by transplantation during growth.
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DOI：
--
发表时间：
1986
期刊：
Birth defects original article series
影响因子：
--
作者：
C. Whitley;N. Ramsay;J. Kersey;W. Krivit
通讯作者：
W. Krivit
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影响因子：
6.2
作者：
R. Recker;D. Kimmel;Michael A. Parfitt;Michael Davies;N. Keshawarz;S. Hinders
通讯作者：
S. Hinders
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DOI：
--
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
猫髂嵴活检部位和腰椎骨小梁重塑测量的组织形态学比较。
影响因子：
1
作者：
Shelley V. Ching;R. Norrdin
通讯作者：
R. Norrdin