Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease. (Q45298923)

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8 January 2020

title

Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease (English)

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Alberto Corsini

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Gillian Bates

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Elena Cattaneo

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Marta Valenza

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Chiara Zuccato

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author name string

Dorotea Rigamonti

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Donato Goffredo

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Simone Fenu

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Laure Jamot

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Andrew Strand

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Alessia Tarditi

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Ben Woodman

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Caterina Mariotti

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Stefano Di Donato

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Rebecca Pruss

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James M Olson

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Simonetta Sipione

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Marzia Tartari

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language of work or name

English

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publication date

1 October 2005

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8 January 2020

published in

Journal of Neuroscience

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volume

25

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8 January 2020

page(s)

9932-9939

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8 January 2020

issue

43

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Apolipoprotein E: from atherosclerosis to Alzheimer's disease and beyond

8 January 2020

cites work

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12 December 2020

Transport of proteins and RNAs in and out of the nucleus

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12 December 2020

Cholesterol binds to synaptophysin and is required for biogenesis of synaptic vesicles

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12 December 2020

Wild-type huntingtin protects from apoptosis upstream of caspase-3.

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12 December 2020

Transcriptional dysregulation in Huntington's disease

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12 December 2020

Smith-Lemli-Opitz syndrome: the first malformation syndrome associated with defective cholesterol synthesis

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12 December 2020

Unsaturated fatty acids down-regulate srebp isoforms 1a and 1c by two mechanisms in HEK-293 cells

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12 December 2020

Loss of normal huntingtin function: new developments in Huntington's disease research

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12 December 2020

Cholesterol metabolism in the brain

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12 December 2020

Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease

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12 December 2020

Protein export from the nucleus

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12 December 2020

Neurobiology. Cholesterol--making or breaking the synapse

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12 December 2020

CNS synaptogenesis promoted by glia-derived cholesterol

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12 December 2020

SREBPs: activators of the complete program of cholesterol and fatty acid synthesis in the liver

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12 December 2020

Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses

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12 December 2020

Cholesterol addition to ER membranes alters conformation of SCAP, the SREBP escort protein that regulates cholesterol metabolism

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12 December 2020

Sterol regulatory element-binding proteins are negatively regulated through SUMO-1 modification independent of the ubiquitin/26 S proteasome pathway

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12 December 2020

Role of cholesterol in synapse formation and function

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12 December 2020

Lipid rafts in the maintenance of synapses, dendritic spines, and surface AMPA receptor stability.

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12 December 2020

Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis

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12 December 2020

Cholesterol homeostasis and function in neurons of the central nervous system

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12 December 2020

Aberrant A2A receptor function in peripheral blood cells in Huntington's disease

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12 December 2020

Combined analysis of oligonucleotide microarray data from transgenic and knockout mice identifies direct SREBP target genes

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12 December 2020

Huntington's disease: a synaptopathy?

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12 December 2020

HMG-CoA reductase inhibition causes neurite loss by interfering with geranylgeranylpyrophosphate synthesis

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12 December 2020

Huntingtin-protein interactions and the pathogenesis of Huntington's disease

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12 December 2020

SUMO modification of Huntingtin and Huntington's disease pathology

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12 December 2020

Selective association of sterol regulatory element-binding protein isoforms with target promoters in vivo

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12 December 2020

Genetic mouse models of Huntington's and Parkinson's diseases: illuminating but imperfect

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12 December 2020

Biomarkers: Potential Uses and Limitations

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12 December 2020

Familial defective apo B-100, characterization of an Italian family

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12 December 2020

Differential loss of striatal projection neurons in Huntington disease

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12 December 2020

Lipophilic beta-adrenoceptor antagonists stimulate cholesterol biosynthesis in human skin fibroblasts

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12 December 2020

Calcium antagonists and low density lipoproteins metabolism by human fibroblasts and by human hepatoma cell line HEP G2

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12 December 2020

In vitro effects of serum proteins and lipids on lipid synthesis in human skin fibroblasts and leukocytes grown in culture

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12 December 2020

Induction of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in human fibroblasts incubated with compactin (ML-236B), a competitive inhibitor of the reductase

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12 December 2020

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

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12 December 2020

Cholesterol for synthesis of myelin is made locally, not imported into brain

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12 December 2020

Cooperation by sterol regulatory element-binding protein and Sp1 in sterol regulation of low density lipoprotein receptor gene

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12 December 2020

SREBP-2, a second basic-helix-loop-helix-leucine zipper protein that stimulates transcription by binding to a sterol regulatory element

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12 December 2020

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

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12 December 2020

Sterol-regulated release of SREBP-2 from cell membranes requires two sequential cleavages, one within a transmembrane segment

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12 December 2020

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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12 December 2020

The SREBP pathway: regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor

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12 December 2020

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

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12 December 2020

Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

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12 December 2020

Cholesterol feeding reduces nuclear forms of sterol regulatory element binding proteins in hamster liver

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12 December 2020

Sterol regulation of 3-hydroxy-3-methylglutaryl-coenzyme A synthase gene through a direct interaction between sterol regulatory element binding protein and the trimeric CCAAT-binding factor/nuclear factor Y.

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12 December 2020

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

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12 December 2020

10.1523/JNEUROSCI.3355-05.2005

Identifiers

DOI

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