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| Content Provider | PubMed Central |
|---|---|
| Author | Abrahamson, M. Grubb, A. |
| Abstract | Hereditary cystatin C amyloid angiopathy is adominantly inherited disorder, characterized by dementia, paralysis, and deathfrom cerebral hemorrhage in early adult life. A variant of the cysteineproteinase inhibitor, cystatin C, is deposited as amyloid in the tissues of thepatients and their spinal-fluid level of cystatin C is abnormally low. Thedisease-associated Leu-68-->Gln mutant (L68Q) cystatin C has been produced inan Escherichia coli expression system and isolated by use of denaturing buffers,immunosorption, and gel filtration. Parallel physicochemical and functionalinvestigations of L68Q-cystatin C and wild-type cystatin C revealed that bothproteins effectively inhibit the cysteine proteinase cathepsin B (equilibriumconstants for dissociation, 0.4 and 0.5 nM, respectively) but differconsiderably in their tendency to dimerize and form aggregates. While wild-typecystatin C is monomeric and functionally active even after prolonged storage atelevated temperatures, L68Q-cystatin C starts to dimerize and lose biologicalactivity immediately after it is transferred to a nondenaturing buffer. Thedimerization of L68Q-cystatin C is highly temperature-dependent, with a rise inincubation temperature from 37 to 40 degrees C resulting in a 150% increase indimerization rate. The aggregation at physiological concentrations is likewiseincreased at 40 compared to 37 degrees C, by approximately 60%. These propertiesof L68Q-cystatin C have bearing upon our understanding of the pathophysiologicalprocess of hereditary cystatin C amyloid angiopathy. They might also be ofclinical relevance, since medical intervention to abort febrile periods ofcarriers of the disease trait may reduce the in vivo formation of L68Q-cystatinC aggregates. |
| Starting Page | 1416 |
| File Format | |
| ISSN | 10916490 |
| e-ISSN | 10916490 |
| Journal | Proceedings of the National Academy of Sciences of the United States of America |
| Issue Number | 4 |
| Volume Number | 91 |
| Language | English |
| Publisher Date | 1994-02-15 |
| Access Restriction | Open |
| Subject Keyword | Research in Higher Education |
| Content Type | Text |
| Resource Type | Article |
| Subject | Multidisciplinary |
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