Two stable unfolding intermediates of the disease-causing L68Q variant of human cystatin C
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 expression system for L68Q cystatin C in Escherichia coli resulting in inclusion body accumulation at a level
