Article
Two stable unfolding intermediates of the disease-causing L68Q variant of human cystatin C.
Biochemistry - 8 Dec 1998
Gerhartz B, Ekiel I, Abrahamson M
Abstract excerpt
In hereditary cystatin C amyloid angiopathy (HCCAA), presence of the Leu68 --> Gln substitution in cystatin C is coupled to a decreased concentration of this major cysteine proteinase inhibitor in cerebrospinal fluid and leads to its amyloid deposition in the brain. We established a high-yield ex...
Topics
- Cerebral Amyloid Angiopathy
- Circular Dichroism
- Cystatin C
- Cystatins
- Dimerization
- Genetic Variation
- Glutamine
- Humans
- Hydrogen-Ion Concentration
- Leucine
- Magnetic Resonance Spectroscopy
- Protein Folding
