PHOSPHOLIPIDS ACCUMULATION IN MUCOLIPIDOSIS-IV CULTURED FIBROBLASTS
PHOSPHOLIPIDS ACCUMULATION IN MUCOLIPIDOSIS-IV CULTURED FIBROBLASTS
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DOI：
10.1007/bf01799863
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发表时间：
1988-01-01
影响因子：
4.2
通讯作者：
BACH, G
中科院分区：
文献类型：
--
作者：
BARGAL, R;BACH, G
培养的成纤维细胞从粘脂沉积症IV患者积累磷脂相比，正常对照组或其他基因型的细胞。主要的储存化合物被确定为磷脂酰胆碱，磷脂酰乙醇胺，并在更大程度上溶血磷脂酰胆碱和lysobisphosphatidic酸。脉冲追逐实验的32 P-标记的磷脂显示增加保留这些化合物在粘脂沉积症IV线在整个脉冲和追逐期间。磷脂酶A1、A2、C、D和溶血磷脂酶在粘脂沉积症IV系中表现出正常活性，因此这种储存的代谢原因仍有待确定。
Cultured fibroblasts from mucolipidosis IV patients accumulated phospholipids when compared to normal controls or cells from other genotypes. The major stored compounds were identified as phosphatidylcholine, phosphatidylethanolamine and to a larger extent lysophosphatidylcholine and lysobisphosphatidic acid. Pulse chase experiments of32P-labelled phospholipids showed increased retention of these compounds in the mucolipidosis IV lines throughout the pulse and chase periods. Phospholipase A1, A2, C, D and lysophospholipase showed normal activity in the mucolipidosis IV lines and thus the metabolic cause for this storage remains to be identified.