Publications related to Major prion protein

Substrate-targeting gamma-secretase modulators

Selective lowering of Abeta42 levels (the 42-residue isoform of the amyloid-beta peptide) with small-molecule gamma-secretase modulators (GSMs), such as some non-steroidal anti-inflammatory drugs, is a promising therapeutic approach for Alzheimer's disease ...

Nature Publishing Group2008

Systemic AAV6 delivery mediating RNA interference against SOD1: neuromuscular transduction does not alter disease progression in fALS mice

Patrick Aebischer, Bernard Schneider, Christopher Towne

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder arising from the selective death of motor neurons. Approximately 20% of familial ALS (fALS) cases are caused by toxic gain-of-function mutations in the superoxide dismutase 1 (SOD1) ...

2008

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host

Peter Frey, Tristan Bolmont, Paolo Paganetti

Protein aggregation is an established pathogenic mechanism in Alzheimer's disease, but little is known about the initiation of this process in vivo. Intracerebral injection of dilute, amyloid-beta (A beta)-containing brain extracts from humans with Alzheim ...

2006

Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro

Steinunn Baekkeskov Hanahan

While a b-sheet-rich form of the prion protein (PrPSc) causes neurodegeneration, the biological activity of its precursor, the cellular prion protein (PrPC), has been elusive. We have studied the effect of purified recombinant prion protein (recPrP) on rat ...

Wiley-Blackwell2005

The materials science of protein aggregation

Hilal Lashuel

Numerous human diseases are associated with conformational change and aggregation of proteins, including Alzheimer's, Parkinson's, prion diseases (such as mad cow disease), familial amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease), Huntington's ...

Materials Research Society2005

Structure and stability of copper-prion protein

Ursula Röthlisberger, Maria Carola Colombo, Leonardo Guidoni

Recent EPR-measurements on the mouse prion protein (mPrP) have indicated that the structured C-terminal domain is capable of binding Cu(II) with high affinity. The structure of Cu(II) binding sites in PrPc are unravelled by exploiting mixed quantum-classic ...

2004

hA molecular switch in amyloid assembly: Met35 and amyloid beta-protein oligomerization

Hilal Lashuel, Sabrina Susan Vollers

Aberrant protein oligomerization is an important pathogenetic process in vivo. In Alzheimer's disease (AD), the amyloid beta-protein (Abeta) forms neurotoxic oligomers. The predominant in vivo Abeta alloforms, Abeta40 and Abeta42, have distinct oligomeriza ...

American Chemical Society2003

The glycan core of GPI-anchored proteins modulates aerolysin binidng but is not sufficient: the polypeptide moiety is required for the toxin-receptor interaction

Françoise Gisou van der Goot Grunberg, Laurence Gouzi Abrami

Sensitivity of mammalian cells to the bacterial toxin aerolysin is due to the presence at their surface of glycosylphosphatidyl inositol (GPI)-anchored proteins which act as receptors. Using a panel of mutants that are affected in the GPI biosynthetic path ...

2002

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Major prion protein

Substrate-targeting gamma-secretase modulators

Systemic AAV6 delivery mediating RNA interference against SOD1: neuromuscular transduction does not alter disease progression in fALS mice

On the structural stability of the free and metal-loaded c-terminal domain of the prion protein

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host

Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro

The materials science of protein aggregation

Molecular electron microscopy approaches to elucidating the mechanisms of protein fibrillogenesis

Structure and stability of copper-prion protein

hA molecular switch in amyloid assembly: Met35 and amyloid beta-protein oligomerization

The glycan core of GPI-anchored proteins modulates aerolysin binidng but is not sufficient: the polypeptide moiety is required for the toxin-receptor interaction

Graph Chatbot

Chat with Graph Search

Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro

Molecular electron microscopy approaches to elucidating the mechanisms of protein fibrillogenesis

Systemic AAV6 delivery mediating RNA interference against SOD1: neuromuscular transduction does not alter disease progression in fALS mice

Substrate-targeting gamma-secretase modulators

On the structural stability of the free and metal-loaded c-terminal domain of the prion protein

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host

The materials science of protein aggregation

Structure and stability of copper-prion protein

hA molecular switch in amyloid assembly: Met35 and amyloid beta-protein oligomerization

The glycan core of GPI-anchored proteins modulates aerolysin binidng but is not sufficient: the polypeptide moiety is required for the toxin-receptor interaction