Publication

Ultrasensitive quantitative measurement of huntingtin phosphorylation at residue S13

Publications associées (32)

One-Pot Semisynthesis of Exon 1 of the Huntingtin Protein: New Tools for Elucidating the Role of Posttranslational Modifications in the Pathogenesis of Huntington's Disease

Giovanni Dietler, Hilal Lashuel, Jae Sun Jeong, Annalisa Ansaloni, Francesco Simone Ruggeri

The natural enzymes involved in regulating many of the posttranslational modifications (PTMs) within the first 17 residues (Nt17) of Huntingtin exon1 (Httex1) remain unknown. A semisynthetic strategy that allows the site-specific introduction of PTMs withi ...

Wiley-Blackwell2014

MicroRNA-22 (miR-22) Overexpression Is Neuroprotective via General Anti-Apoptotic Effects and May also Target Specific Huntington's Disease-Related Mechanisms

Ana Jovicic

Background: Whereas many causes and mechanisms of neurodegenerative diseases have been identified, very few therapeutic strategies have emerged in parallel. One possible explanation is that successful treatment strategy may require simultaneous targeting o ...

Public Library Science2013

Phosphorylation of 4E-BP1 in the Mammalian Brain Is Not Altered by LRRK2 Expression or Pathogenic Mutations

Liliane Glauser, Klodjan Stafa, Darren Moore, Alzbeta Trancikova, Elpida Tsika

Mutations in the leucine-rich repeat kinase 2 (LRRK2) gene are a common cause of autosomal dominant familial Parkinson's disease (PD). LRRK2 encodes a multi-domain protein containing GTPase and kinase enzymatic domains. Disease-associated mutations in LRRK ...

Public Library of Science2012

Quantitative fluorescence loss in photobleaching for analysis of protein transport and aggregation

Daniel Sage

Background: Fluorescence loss in photobleaching (FLIP) is a widely used imaging technique, which provides information about protein dynamics in various cellular regions. In FLIP, a small cellular region is repeatedly illuminated by an intense laser pulse, ...

Biomed Central Ltd2012

Gene expression profiling of R6/2 transgenic mice with different CAG repeat lengths reveals genes associated with disease onset and progression in Huntington's disease

R6/2 transgenic mice with expanded CAG repeats (> 300) have a surprisingly prolonged disease progression and longer lifespan than prototypical parent R6/2 mice (carrying 150 CAGs); however, the mechanism of this phenotype amelioration is unknown. We compar ...

Elsevier2011

Synchrotron Infrared Microspectroscopy Detecting the Evolution of Huntington's Disease Neuropathology and Suggesting Unique Correlates of Dysfunction in White versus Gray Brain Matter

László Forró, Sylvia Jeney, Heike Runne, Markus Bonda

Huntington's disease (HD), caused by a mutation of the corresponding gene encoding the protein huntingtin (htt), is characterized by progressive deterioration of cognitive and motor functions, paralleled by extensive loss of striatal neurons. At the cellul ...

2011

Diminished activity-dependent brain-derived neurotrophic factor expression underlies cortical neuron microcircuit hypoconnectivity resulting from exposure to mutant huntingtin fragments

Luca Gambazzi, Henry Markram, Heike Runne, Ozgün Gökce, Michele Giugliano, Elena Katsyuba

Although previous studies of Huntington's disease (HD) have addressed many potential mechanisms of striatal neuron dysfunction and death, it is also known, based on clinical findings, that cortical function is dramatically disrupted in HD. With respect to ...

American Society for Pharmacology and Experimental Therapeutics2010

Sustained Effects of Nonallele-Specific Huntingtin Silencing

Nicole Déglon

Objective: Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin (htt) protein. No cure is available to date to alleviate neurodegeneration. Recent studies have demonstrated ...

Wiley-Blackwell2009

Implication of the JNK pathway in a rat model of Huntington's disease

Patrick Aebischer, Nicole Déglon

Huntington's disease (HD) is a neurodegenerative disorder resulting from the expansion of a glutamine repeat (polyQ) in the N-terminus of the huntingtin (htt) protein. Expression of polyQ-containing proteins has been previously shown to induce various cell ...

2009

Effects of BDNF and other neuroprotectants on the neuropathology of Huntington's disease

Ozgün Gökce

Huntington's disease is an autosomal dominantly inherited neurodegenerative disorder characterized by progressive motor dysfunction, dementia, psychiatric symptoms, and weight loss, eventually leading to death. Postmortem analysis of the brains of HD patie ...

EPFL2009

Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons

Nicole Déglon, Ozgün Gökce, Nikita Rudinskiy

Hippocalcin is a neuronal calcium sensor protein previously implicated in regulating neuronal viability and plasticity. Hippocalcin is the most highly expressed neuronal calcium sensor in the medium spiny striatal output neurons that degenerate selectively ...

Wiley-Blackwell2009

Transcriptional dysregulation in Huntington's disease

Heike Runne

Huntington's disease (HD) is a progressive neurodegenerative disorder with autosomal dominant inheritance. It is caused by a singular mutation in exon 1 of the HD gene encoding an abnormal polyglutamine (polyQ) expansion in the N-terminal region of the hun ...

EPFL2008