Sökning: "Frataxin"

Hittade 5 avhandlingar innehållade ordet Frataxin.

1. 1. Oligomerization of human and bacterial frataxin : Structural and functional studies

   Författare :Mostafa Abdalkhalik; Biokemi och Strukturbiologi; []
   Nyckelord :Frataxin; Friedreich’s ataxia; iron; reactive oxiden species; protein oligomerization; SAXS; EM; cross-linking; XAFS; nano- DSF;

   Sammanfattning : Friedreich’s ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by defeciency in frataxin, a highly conserved protein central to iron homeostasis in mirochondria. The function of frataxin is related to its capability to bind, store and deliver iron to different biochemical process such as iron-sulfur cluster assembly and heme biosynthesis. LÄS MER

3. 2. The complex world of proteins: Structure, function, and oligomerization of frataxin

   Författare :Christopher Söderberg; Biokemi och Strukturbiologi; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; Frataxin; Friedreich s ataxia; self-assembly; protein-protein interaction; iron homeostasis; SAXS; cross-linking;

   Sammanfattning : Patients suffering from the deadly progressive neurodegenerative disease Friedreich's ataxia have reduced levels of expression of the protein frataxin. Yeast frataxin has been extensively studied in vitro, and has been shown to deliver iron to heme and iron-sulfur cluster biosynthesis, as well as to oligomerize in the presence of iron. LÄS MER

4. 3. Iron in Haem Biosynthesis. Structural Studies of Ferrochelatase and Frataxin

   Författare :Tobias Karlberg; Biokemi och Strukturbiologi; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; Naturvetenskap; Natural science; Molekylär biofysik; Molecular biophysics; Biomedicinska vetenskaper; Biomedical sciences; protein crystallography; ferrochelatase; haem biosynthesis; iron; frataxin;

   Sammanfattning : Ferrochelatase catalyses the synthesis of heme by inserting Fe(II) into protoporphyrin IX. The structure of ferrochelatase from yeast has been determined (2.4 Å). The structure is homodimeric with each of the monomer made up of two Rossmann type domains with an active-site cleft between them. LÄS MER

5. 4. Structural Insights into the Acquisition and Storage of Iron

   Författare :Ulrika Schagerlöf; Biokemi och Strukturbiologi; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; Strukturkemi; Molecular biophysics; Molekylär biofysik; Structure chemistry; single particle; ferric reductase; human phagocyte superoxide-generating NADPH oxidase; frataxin; cryo electron microscopy; FRO2; iron homeostasis; iron acquisition;

   Sammanfattning : It is of vital importance to understand the systems for iron metabolism and this thesis contributes to this by providing insights into the structure and function of the acquisition and homeostasis of iron. The two proteins that have been primarily investigated are a ferric reductase from Arabidopsis thaliana called FRO2 and a mitochondrial iron chaperone from Saccharomyces cerevisiae called frataxin. LÄS MER

7. 5. Development of benzoquinoquinoxaline derivatives as triplex-specific probes : Recognition of DNA structures at repeats sequences

   Författare :Helen Bergquist; Rula Zain; Keith Fox; Stockholms universitet; []
   Nyckelord :NATURVETENSKAP; NATURAL SCIENCES; Triplex; H-DNA; BQQ; BQQ-OP; BQQ-Bodipy; triplet repeat; DNA; non-B-DNA; pkd1; frataxin; Friedreich s ataxia; Molecular biology; Molekylärbiologi; Molecular Biology; molekylärbiologi;

   Sammanfattning : Repeat sequences are associated with several human diseases, such as Friedreich’s ataxia, polycystic kidney disease and cancer. These sequences can form non-B-DNA structures, including triplex (H-DNA) DNA, and are associated with genomic instability and altered gene expression. LÄS MER