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Research area/areas: Biomedical Laboratory Science/Technology, Clinical Laboratory Medicine, General Medicine, Medicinal Chemistry, Microbiology in the medical area, Other Clinical Medicine
We sequenced in 1981 a protein with unknown function, now called cystatin C, which defined a new superfamily of cysteine protease inhibitors ("cystatins"). We have described several new cystatins and shown that cystatin C is produced by a house-keeping gene and that a mutation in it causes the dominantly inherited disease "Hereditary Cystatin C Amyloid Angiopathy" in which L68Q-cystatin C is precipitated as amyloid in affected individuals, who die from brain hemorrhage before 40 years of age. We have demonstrated that cystatin C amyloid deposits may form by "propagated domain-swapping" and shown that inhibition of domain swapping prevents the in vitro formation of cystatin C amyloid. We have also demonstrated that cystatin C modulates the amyloid deposits in Alzheimer disease. We have demonstrated that certain peptidyl derivatives based upon the structure of cystatin C will kill grampositive bacteria resistant to all known presently available antibiotic compounds and some viruses.
We sequenced in 1981 a protein with unknown function, now called cystatin C, which defined a new superfamily of cysteine protease inhibitors ("cystatins"). We have described several new cystatins and shown that cystatin C is produced by a house-keeping gene and that a mutation in it causes the dominantly inherited disease "Hereditary Cystatin C Amyloid Angiopathy" in which L68Q-cystatin C is precipitated as amyloid in affected individuals, who die from brain hemorrhage before 40 years of age. We have demonstrated that cystatin C amyloid deposits may form by "propagated domain-swapping" and shown that inhibition of domain swapping prevents the in vitro formation of cystatin C amyloid. We have also demonstrated that cystatin C modulates the amyloid deposits in Alzheimer disease. We have demonstrated that certain peptidyl derivatives based upon the structure of cystatin C will kill grampositive bacteria resistant to all known presently available antibiotic compounds and some viruses.
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Johann Sigurjonsson, David Grubb,Anders Grubb,Anders Christensson,Carl M Öberg,Per Ederoth,Sasha Koul,Matthias Götberg,Troels Yndigegn, Tim Tödt, Benedicte Viterius,Henrik Bjursten
American journal of kidney diseases : the official journal of the National Kidney Foundation (2024)
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Nephrology Dialysis Transplantationno. Supplement_1 (2024)
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGYno. 12 (2023): 1953-1964
Kidney international reportsno. 8 (2023): 1672-1675
American journal of kidney diseases : the official journal of the National Kidney Foundationno. 5 (2023): 509-511
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