STORAGE OF SAPOSIN-A AND SAPOSIN-D IN INFANTILE NEURONAL CEROID-LIPOFUSCINOSIS

被引：158

作者：

TYYNELA, J

PALMER, DN

BAUMANN, M

HALTIA, M

机构：

[1] UNIV HELSINKI, DEPT PATHOL, HAARTMANINKATU 3, SF-00290 HELSINKI 29, FINLAND

[2] UNIV HELSINKI, DEPT MED CHEM, SF-00014 HELSINKI, FINLAND

[3] MASSEY UNIV, DEPT VET PATHOL & PUBL HLTH, PALMERSTON NORTH, NEW ZEALAND

来源：

FEBS LETTERS | 1993年 / 330卷 / 01期

关键词：

INFANTILE NEURONAL CEROID-LIPOFUSCINOSIS; SAPOSIN; LYSOSOMAL STORAGE DISEASE; BATTEN DISEASE;

D O I：

10.1016/0014-5793(93)80908-D

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

We have isolated storage cytosomes from brain tissue of patients with infantile neuronal ceroid-lipofuscinosis. The purified storage bodies were subjected to compositional analysis which revealed a high content of proteins, accounting for 43% of dry weight. Saposins A and D, also known as sphingolipid activator proteins (SAPs), were shown to constitute a major portion of the accumulated protein using gel electrophoresis and sequence analysis. This is the first time that saposins have been found to be stored in any form of neuronal ceroid-lipofuscinosis.

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页码：8 / 12

页数：5

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