A fully humanized transgenic mouse model of Huntington disease. (Q30537951)

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8 March 2020

title

A fully humanized transgenic mouse model of Huntington disease (English)

1 reference

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8 March 2020

0 references

1

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8 March 2020

Jeffrey B. Carroll

3

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8 March 2020

Niels Skotte

5

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8 March 2020

Erika B Villanueva

7

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8 March 2020

Mahmoud A. Pouladi

10

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8 March 2020

Michael R. Hayden

14

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8 March 2020

author name string

Simon C Warby

2

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8 March 2020

Crystal N Doty

4

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8 March 2020

Weining Zhang

6

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8 March 2020

Vlad Kovalik

8

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8 March 2020

Yuanyun Xie

9

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8 March 2020

Jennifer A Collins

11

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8 March 2020

X William Yang

12

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8 March 2020

Sonia Franciosi

13

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publication date

21 September 2012

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8 March 2020

published in

Human Molecular Genetics

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8 March 2020

volume

22

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8 March 2020

issue

1

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8 March 2020

page(s)

18-34

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Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis.

8 March 2020

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Preclinical safety of RNAi-mediated HTT suppression in the rhesus macaque as a potential therapy for Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

A natural antisense transcript at the Huntington's disease repeat locus regulates HTT expression

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Huntington's disease - neuropathology

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Mutant huntingtin causes metabolic imbalance by disruption of hypothalamic neurocircuits

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Huntington's disease: from molecular pathogenesis to clinical treatment

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Sustained effects of nonallele-specific Huntingtin silencing

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

CAG expansion in the Huntington disease gene is associated with a specific and targetable predisposing haplogroup

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

CAG-encoded polyglutamine length polymorphism in the human genome

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Full motor recovery despite striatal neuron loss and formation of irreversible amyloid-like inclusions in a conditional mouse model of Huntington's disease.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Mouse Huntington's disease gene homolog (Hdh).

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3606012

Lessons from predictive testing for Huntington disease: 25 years on.

20 June 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

rAAV-mediated shRNA ameliorated neuropathology in Huntington disease model mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

Body weight is modulated by levels of full-length huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDS397

21 January 2018

Identifiers

DOI

10.1093/HMG/DDS397

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:23001568%20AND%20SRC:MED&resulttype=core&format=json

8 March 2020

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:23001568%20AND%20SRC:MED&resulttype=core&format=json

8 March 2020

PubMed publication ID

23001568

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:23001568%20AND%20SRC:MED&resulttype=core&format=json