Evidence map›Paper›PMID 35023827›Full record

ArticleeLife2022

A novel and accurate full-length HTT mouse model for Huntington's disease.

Sushila A Shenoy, Sushuang Zheng, Wencheng Liu, Yuanyi Dai, Yuanxiu Liu, Zhipeng Hou, Susumu Mori, Yi Tang, Jerry Cheng, Wenzhen Duan and 1 more

Open access · goldAbstract read
In one paragraph

Article in eLife, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.

0numbers the graph read from it
0cells of the map it votes in
18citing papers in PubMed
2.5field-weighted citation impact, top 10% 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

18 citing papers in PubMed, 29 citations in OpenAlex.

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  5. Animal models of Huntington's disease. Pros and cons.Brain, behavior, & immunity - health · 2025
    Review
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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

11 authors at 5 institutions in 2 countries.

Sushila A Shenoy *Department of Neuroscience, Weill Cornell Graduate School of Medical Sciences, New York, United States.
Sushuang Zheng *The MOE Key Laboratory of Cell Proliferation and Differentiation, School of Life Sciences, Peking University, Beijing, China.
Wencheng LiuDepartment of Neuroscience, Weill Cornell Graduate School of Medical Sciences, New York, United States.
Yuanyi DaiThe MOE Key Laboratory of Cell Proliferation and Differentiation, School of Life Sciences, Peking University, Beijing, China.
Yuanxiu LiuThe MOE Key Laboratory of Cell Proliferation and Differentiation, School of Life Sciences, Peking University, Beijing, China.
Zhipeng HouThe Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, United States.
Susumu MoriThe Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, United States.
Yi TangInnovation Center for Neurological Disorders, Department of Neurology, Xuanwu Hospital Capital Medical University, National Center for Neurological Disorders, Beijing, China.
Jerry ChengDepartment of Computer Science, New York Institute of Technology, New York, United States.
Wenzhen DuanDivision of Neurobiology, Department of Psychiatry and Behavioral Sciences; Solomon H.Snyder Department of Neuroscience, Johns Hopkins University School of medicine, Baltimore, United States.
Chenjian LiThe MOE Key Laboratory of Cell Proliferation and Differentiation, School of Life Sciences, Peking University, Beijing, China.ORCID 0000-0001-7905-6434
Peking University · CNJohns Hopkins University · USCornell University · USCapital Medical University · CNNew York Institute of Technology · US

Funding

Advanced MRI biomarkers in HD mouse models translatable to humans: nature history and response to therapeuticsR01NS124084 · NINDS · JOHNS HOPKINS UNIVERSITY · PI DUAN, WENZHEN · 2022 to 2025
$2.6M
Huntington's Disease biomarkers and therapeuticsR01NS082338 · NINDS · JOHNS HOPKINS UNIVERSITY · PI DUAN, WENZHEN · 2013 to 2017
$2.0M
Structure of Triplet Repeat mRNA in Neurodegenerative DiseaseR01NS099397 · NINDS · JOHNS HOPKINS UNIVERSITY · PI DUAN, WENZHEN, WOODSON, SARAH A. · 2016 to 2020
$1.8M
Advanced MRI biomarkers in HD mouse models translatable to humans: nature history and response to therapeuticsR56NS124084 · NINDS · JOHNS HOPKINS UNIVERSITY · PI DUAN, WENZHEN · 2021 to 2021
$522k
Imaging brain glucose uptake by onVDMP MRI in Huntington's DiseaseR21NS118079 · NINDS · JOHNS HOPKINS UNIVERSITY · PI DUAN, WENZHEN, XU, JIADI · 2020 to 2020
$465k
Cerebrovascular Abnormalities in Huntington's DiseaseR21NS104480 · NINDS · HUGO W. MOSER RES INST KENNEDY KRIEGER · PI DUAN, WENZHEN, HUA, JUN · 2018 to 2019
$446k
NINDS NIH HHS R01 NS082338NINDS NIH HHS R21 NS118079NINDS NIH HHS R56 NS124084
6 · The paper itself

Abstract

Here, we report the generation and characterization of a novel Huntington's disease (HD) mouse model BAC226Q by using a bacterial artificial chromosome (BAC) system, expressing full-length human HTT with ~226 CAG-CAA repeats and containing endogenous human HTT promoter and regulatory elements. BAC226Q recapitulated a full-spectrum of age-dependent and progressive HD-like phenotypes without unwanted and erroneous phenotypes. BAC226Q mice developed normally, and gradually exhibited HD-like psychiatric and cognitive phenotypes at 2 months. From 3 to 4 months, BAC226Q mice showed robust progressive motor deficits. At 11 months, BAC226Q mice showed significant reduced life span, gradual weight loss and exhibited neuropathology including significant brain atrophy specific to striatum and cortex, striatal neuronal death, widespread huntingtin inclusions, and reactive pathology. Therefore, the novel BAC226Q mouse accurately recapitulating robust, age-dependent, progressive HD-like phenotypes will be a valuable tool for studying disease mechanisms, identifying biomarkers, and testing gene-targeting therapeutic approaches for HD.

Indexed as

Disease Models, AnimalHuntingtin ProteinHuntington DiseaseAnimalsChromosomes, Artificial, BacterialFemaleHumansMaleMiceMice, TransgenicHTT protein, humanHuntingtin Proteinbody weight lossbrain atrophyhtt aggregatesHtt aggregateshuman bachuntington's diseasehuntington’s diseaseneurodegenerationneuron lossneurosciencetransgenic mouse

Identifiers

PMID35023827
PMCPMC8758142
OpenAlexW4206688506

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.