Sökning: id:"swepub:oai:DiVA.org:liu-19548" >
Small-Molecule Supp...
Small-Molecule Suppression of Misfolding of Mutated Human Carbonic Anhydrase II Linked to Marble Brain Disease
-
- Almstedt, Karin (författare)
- Linköpings universitet,Biokemi,Tekniska högskolan
-
- Rafstedt, Therese (författare)
- Linköpings universitet,Institutionen för fysik, kemi och biologi,Tekniska högskolan
-
- Supuran, Claudiu T (författare)
- University of Florence
-
visa fler...
-
- Carlsson, Uno (författare)
- Linköpings universitet,Biokemi,Tekniska högskolan
-
- Hammarström, Per (författare)
- Linköpings universitet,Biokemi,Tekniska högskolan
-
visa färre...
-
(creator_code:org_t)
- 2009-05-20
- 2009
- Engelska.
-
Ingår i: Biochemistry. - : American Chemical Society (ACS). - 0006-2960 .- 1520-4995. ; 48:23, s. 5358-5364
- Relaterad länk:
-
http://urn.kb.se/res...
-
visa fler...
-
https://urn.kb.se/re...
-
https://doi.org/10.1...
-
visa färre...
Abstract
Ämnesord
Stäng
- Carbonic anhydrase II deficiency syndrome or Marble brain disease (MBD) is caused by autosomal recessive mutations in the human carbonic anhydrase II (HCA II) gene. Here we report a small-molecule stabilization study of the exceptionally destabilized HCA II mutant H107Y employing inhibitors based on p-aminobenzoyisulfonamide compounds and 1,3,4-thiadiazolylsulfonamides as well as amino acid activators. Protein stability assays showed a significant stabilization by the aromatic sulfonamide inhibitors when present at 10 mu M concentration, providing shifts of the midpoint of thermal denaturation between 10 degrees C and 16 degrees C and increasing the free energies of denaturation 0.5-3.0 kcal/mol as deduced from GuHCl denaturation. This study could be used as a starting point for the design of small-molecule folding modulators and possibly autoactivatable molecules for suppression of misfolding of destabilized HCA II mutants.
Nyckelord
- NATURAL SCIENCES
- NATURVETENSKAP
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
Hitta via bibliotek
Till lärosätets databas