Storage of saposins A and D in infantile neuronal ceroid‐lipofuscinosis

FEBS Letters

Tyynelä, Jaana²  Palmer, David N.¹  Baumann, Marc²  Haltia, Matti³ 

[1] Department of Veterinary Pathology and Public Health, Massey University, Palmerston North, New Zealand;Department of Medical Chemistry, PO Box 8, University of Helsinki, SF-00014 Helsinki, Finland;Department of Pathology, University of Helsinki, Haartmaninkatu 3, SF-00290 Helsinki, Finland

关键词: Infantile neuronal ceroid-lipofuscinosis;    Saposin;    Lysosomal storage disease;    Batten's disease;    GFAP;    glial fibrillary acidic protein;    INCL;    infantile neuronal ceroid-lipofuscinosis;    JNCL;    juvenile neuronal ceroid-lipofuscinosis;    LDS;    lithium dodecyl sulphate;    LINCL;    late infantile neuronal ceroid-lipofuscinosis;    NCL;    neuronal ceroid-lipofuscinosis;   

DOI  :  10.1016/0014-5793(93)80908-D

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 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.

【 授权许可】

Unknown