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Molecular Mechanisms Underlying Hyaline Fibromatosis Syndrome

Publications associées (46)

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Peptide ligands for targeting and retention of nanoparticles and protein therapeutics in articular cartilage

Dominique André Rothenfluh

Osteoarthritis (OA) is one of the most common causes of pain and disability. Currently, non-surgical treatment is limited to analgesic and anti-inflammatory drugs, which are not capable of either modifying the disease process or regenerate damaged parts in ...

EPFL2009

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Systemic hyalinosis is an autosomal recessive disease that encompasses two allelic syndromes, infantile systemic hyalinosis (ISH) and juvenile hyaline fibromatosis (JHF), which are caused by mutations in the CMG2 gene. Here we have analyzed the cellular co ...

Wiley-Blackwell2009

Receptors of anthrax toxin and cell entry

Françoise Gisou van der Goot Grunberg

Anthrax toxin-receptor interactions are critical for toxin delivery to the host cell cytoplasm. This review summarizes what is known about the molecular details of the protective antigen (PA) toxin subunit interaction with either the ANTXR1 and ANTXR2 cell ...

Elsevier2009

Most tissue engineering approaches include the delivery of reparative cells to a damaged host tissue. Generally these cells are sought to be of a stem cell character since they retain a high potential for proliferation and differentiation into diverse phen ...

EPFL2008

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To exert its activity, anthrax toxin must be endocytosed and its enzymatic toxic subunits delivered to the cytoplasm. It has been proposed that, in addition to the anthrax toxin receptors (ATRs), lipoprotein-receptor-related protein 6 (LRP6), known for its ...

2008

Molecular heterogeneity of the ganglionic eminence and its relationship to the birth of neocortical interneurons

Susan Willi Monnerat

Neocortical function and malfunction depends critically on a spectrum of inhibitory interneurons. To gain novel insight in normal brain function and mechanisms leading to diseases, such as epilepsy or schizophrenia, the knowledge about the specific functio ...

EPFL2007

Glycosylation pathways as drug targets for cancer: glycosidase inhibitors

Sandrine Gerber

The combined and ordered sequential action of glycosidases and glycosyltransferases in mammalian cell compartments leads to the addition of defined glycans to proteins and lipids. Altered glycosylation patterns, neoexpression, underexpression or overexpres ...

2006

Anthrax toxin: the long and winding road that leads to the kill

Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami

The past five years have led to a tremendous increase in our molecular understanding of the mode of action of the anthrax toxin, one of the two main virulence factors produced by Bacillus anthracis. The structures of each of the three components of the tox ...

Elsevier2005

Gpi8p and Gaa1p are essential components of the GPI transamidase that adds glycosylphosphatidylinositols (GPIs) to newly synthesized proteins. After solubilization in 1.5% digitonin and separation by blue native PAGE, Gpi8p is found in 430-650-kDa protein ...

American Society for Cell Biology2001

Synthesis of biotinylated glycosulfopeptides by chemoselective ligation

Patricia Durieux

Tyrosine sulfation and O-glycosylation are two post-translational modifications playing an essential role in modulation of biol. activity, protein folding and cellular communication. Here, a novel chem. approach for the synthesis of biotinylated glycosulfo ...

2001