Evidence map›Paper›PMID 20154343›Full record

ArticleHuman molecular genetics2010

An ovine transgenic Huntington's disease model.

Jessie C Jacobsen, C Simon Bawden, Skye R Rudiger, Clive J McLaughlan, Suzanne J Reid, Henry J Waldvogel, Marcy E MacDonald, James F Gusella, Simon K Walker, Jennifer M Kelly and 4 more

Open access · bronzeAbstract read
In one paragraph

Article in Human molecular genetics, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 84 papers.

0numbers the graph read from it
0cells of the map it votes in
84citing papers in PubMed
4.5field-weighted citation impact, top 4% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

84 citing papers in PubMed, 187 citations in OpenAlex.

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24 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

14 authors at 6 institutions in 4 countries.

Jessie C JacobsenDepartment of Molecular Medicine and Pathology, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand.
C Simon Bawden
Skye R Rudiger
Clive J McLaughlan
Suzanne J Reid
Henry J Waldvogel
Marcy E MacDonald
James F Gusella
Simon K Walker
Jennifer M Kelly
Graham C Webb
Richard L M Faull
Mark I Rees
Russell G Snell
South Australian Research and Development Institute · AUUniversity of Auckland · NZCardiff University · GBCenter for Human Genetics · USMassachusetts General Hospital · USUniversity of Adelaide · AU

Funding

TOXICITY AND SPECIFICITY COMPONENTS OF HD PATHOGENESISP50NS016367 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI MACDONALD, MARCY · 1996 to 2010
$15.8M
GENETIC MODEL OF HUNTINGTONS DISEASER01NS032765 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI MACDONALD, MARCY · 1994 to 2011
$3.7M
RECEPTOR MECHANISMS IN STRIATAL DEVELOPMENT AND HUNTINGTON'S DISEASEP01NS016367 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI GUSELLA, JAMES F · 1985 to 1995
–
NINDS NIH HHS NS16367NINDS NIH HHS NS32765NINDS NIH HHS P50 NS016367NINDS NIH HHS R01 NS032765
6 · The paper itself

Abstract

Huntington's disease (HD) is an inherited autosomal dominant neurodegenerative disorder caused by an expansion of a CAG trinucleotide repeat in the huntingtin (HTT) gene [Huntington's Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell, 72, 971-983]. Despite identification of the gene in 1993, the underlying life-long disease process and effective treatments to prevent or delay it remain elusive. In an effort to fast-track treatment strategies for HD into clinical trials, we have developed a new large-animal HD transgenic ovine model. Sheep, Ovis aries L., were selected because the developmental pattern of the ovine basal ganglia and cortex (the regions primarily affected in HD) is similar to the analogous regions of the human brain. Microinjection of a full-length human HTT cDNA containing 73 polyglutamine repeats under the control of the human promotor resulted in six transgenic founders varying in copy number of the transgene. Analysis of offspring (at 1 and 7 months of age) from one of the founders showed robust expression of the full-length human HTT protein in both CNS and non-CNS tissue. Further, preliminary immunohistochemical analysis demonstrated the organization of the caudate nucleus and putamen and revealed decreased expression of medium size spiny neuron marker DARPP-32 at 7 months of age. It is anticipated that this novel transgenic animal will represent a practical model for drug/clinical trials and surgical interventions especially aimed at delaying or preventing HD initiation. New sequence accession number for ovine HTT mRNA: FJ457100.

Indexed as

Disease Models, AnimalAnimalsAnimals, Genetically ModifiedBasal GangliaChromosomes, MammalianDopamine and cAMP-Regulated Phosphoprotein 32FemaleFounder EffectHumansHuntingtin ProteinHuntington DiseaseMaleNerve Tissue ProteinsNuclear ProteinsPedigreeReceptor, Cannabinoid, CB1Dopamine and cAMP-Regulated Phosphoprotein 32HTT protein, humanHuntingtin ProteinNerve Tissue ProteinsNuclear ProteinsReceptor, Cannabinoid, CB1

Identifiers

PMID20154343
PMCPMC2860888
OpenAlexW2138833649

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.