A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths. (Q64882921)

30 June 2019

title

A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths. (English)

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Tammy Gillis

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object named as

Tammy Gillis

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author name string

Claudia Lin-Kar Hung

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Laura Erin Bowie

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Ryan Gotesman

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Susie Son

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Mina Falcone

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James Victor Giordano

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Virginia Mattis

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Trevor Lau

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Vickie Kwan

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Vanessa Wheeler

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Jonathan Schertzer

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Karun Singh

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Ray Truant

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publication date

26 September 2018

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Molecular Biology of the Cell

published in

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volume

29

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issue

23

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page(s)

2809-2820

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describes a project that uses

ImageJ

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC6249865/fullTextXML

inferred from PubMed Central ID database lookup

cites work

Anomalous cellular proliferation in vitro associated with Huntington's disease

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12 December 2020

Abnormalities in growth of skin fibroblasts of patients with Huntington's disease

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12 December 2020

Fibroblast cultures in Huntington's disease

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12 December 2020

Senescence of cultured human fibroblasts: mitotic versus metabolic time.

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12 December 2020

Skin fibroblasts in Huntington disease.

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A biomarker that identifies senescent human cells in culture and in aging skin in vivo

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Involvement of the p53 tumor suppressor in repair of u.v.-type DNA damage.

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12 December 2020

Somatic and gonadal mosaicism of the Huntington disease gene CAG repeat in brain and sperm.

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12 December 2020

A new polymerase chain reaction (PCR) assay for the trinucleotide repeat that is unstable and expanded on Huntington's disease chromosomes

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12 December 2020

Induction of nuclear accumulation of the tumor-suppressor protein p53 by DNA-damaging agents

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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

The role of p53 in cell cycle regulation

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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

Extension of life-span by introduction of telomerase into normal human cells

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12 December 2020

Reconstitution of telomerase activity in normal human cells leads to elongation of telomeres and extended replicative life span

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12 December 2020

Telomerase expression in human somatic cells does not induce changes associated with a transformed phenotype

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12 December 2020

Absence of cancer-associated changes in human fibroblasts immortalized with telomerase

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12 December 2020

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

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12 December 2020

A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice

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12 December 2020

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

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12 December 2020

The establishment of telomerase-immortalized cell lines representing human chromosome instability syndromes

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12 December 2020

Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells

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Neurological abnormalities in a knock-in mouse model of Huntington's disease

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Characterization of ataxia telangiectasia fibroblasts with extended life-span through telomerase expression.

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p53 protein at the hub of cellular DNA damage response pathways through sequence-specific and non-sequence-specific DNA binding

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12 December 2020

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

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12 December 2020

Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment.

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12 December 2020

Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with beta-tubulin: relevance to Huntington's disease

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12 December 2020

Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease

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12 December 2020

Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats

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12 December 2020

The serial cultivation of human diploid cell strains

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12 December 2020

ENDOREDUPLICATION IN HUMAN FIBROBLAST CULTURES

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12 December 2020

THE LIMITED IN VITRO LIFETIME OF HUMAN DIPLOID CELL STRAINS

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12 December 2020

Alterations in the p16(INK4a) and p53 tumor suppressor genes of hTERT-immortalized human fibroblasts.

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12 December 2020

Senescent fibroblasts resist apoptosis by downregulating caspase-3.

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12 December 2020

Huntington's disease genetics

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12 December 2020

Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin

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12 December 2020

p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease

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12 December 2020

Continuous growth of telomerase-immortalised fibroblasts: how long do cells remain normal?

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12 December 2020

p53 tumor suppressor protein regulates the levels of huntingtin gene expression

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12 December 2020

Body weight is modulated by levels of full-length huntingtin

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12 December 2020

Molecular anatomy of a speckle

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12 December 2020

Oxidative stress in skin fibroblasts cultures of patients with Huntington's disease.

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12 December 2020

Human diploid fibroblast cells in senescence; cycling through polyploidy to mitotic cells

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12 December 2020

UVA irradiation induces relocalisation of the DNA repair protein hOGG1 to nuclear speckles

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12 December 2020

Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity

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12 December 2020

A stress sensitive ER membrane-association domain in Huntingtin protein defines a potential role for Huntingtin in the regulation of autophagy

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12 December 2020

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

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12 December 2020

The splicing factor SC35 has an active role in transcriptional elongation

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12 December 2020

Use of exogenous hTERT to immortalize primary human cells

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12 December 2020

Protocols to detect senescence-associated beta-galactosidase (SA-betagal) activity, a biomarker of senescent cells in culture and in vivo

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12 December 2020

Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice

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12 December 2020

The transcriptional programme of contact-inhibition

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12 December 2020

Quiescent fibroblasts exhibit high metabolic activity.

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12 December 2020

Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease

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12 December 2020

Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis

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12 December 2020

Kinase inhibitors modulate huntingtin cell localization and toxicity.

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12 December 2020

MicroRNA-mediated conversion of human fibroblasts to neurons.

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12 December 2020

Role of cell cycle re-entry in neurons: a common apoptotic mechanism of neuronal cell death

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12 December 2020

The p53 network: cellular and systemic DNA damage responses in aging and cancer

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12 December 2020

Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice

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12 December 2020

Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy

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12 December 2020

Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer

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12 December 2020

Generation and applications of human pluripotent stem cells induced into neural lineages and neural tissues

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12 December 2020

Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells

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12 December 2020

Cofilin nuclear-cytoplasmic shuttling affects cofilin-actin rod formation during stress

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12 December 2020

Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes

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12 December 2020

Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington's disease

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12 December 2020

Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175.

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12 December 2020

The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal

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12 December 2020

Direct conversion of fibroblasts to neurons by reprogramming PTB-regulated microRNA circuits

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12 December 2020

The genomic and transcriptomic landscape of a HeLa cell line

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12 December 2020

The fractured genome of HeLa cells

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12 December 2020

Aneuploidy and chromosomal instability: a vicious cycle driving cellular evolution and cancer genome chaos

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12 December 2020

Rapid single-step induction of functional neurons from human pluripotent stem cells

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12 December 2020

A failure in energy metabolism and antioxidant uptake precede symptoms of Huntington's disease in mice.

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12 December 2020

Studies on the Q175 Knock-in Model of Huntington's Disease Using Functional Imaging in Awake Mice: Evidence of Olfactory Dysfunction

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12 December 2020

A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease.

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12 December 2020

Generation of human striatal neurons by microRNA-dependent direct conversion of fibroblasts

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12 December 2020

Identification of Genetic Factors that Modify Clinical Onset of Huntington's Disease

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12 December 2020

Long Term Aggresome Accumulation Leads to DNA Damage, p53-dependent Cell Cycle Arrest, and Steric Interference in Mitosis.

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12 December 2020

Effects of aneuploidy on gene expression: implications for cancer

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12 December 2020

Disease-associated repeat instability and mismatch repair

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12 December 2020

The HTT CAG-Expansion Mutation Determines Age at Death but Not Disease Duration in Huntington Disease

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12 December 2020

An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes

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12 December 2020

Huntingtin N17 domain is a reactive oxygen species sensor regulating huntingtin phosphorylation and localization

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12 December 2020

Huntingtin is a scaffolding protein in the ATM oxidative DNA damage response complex.

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12 December 2020

Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease.

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12 December 2020

Striatal neurons directly converted from Huntington's disease patient fibroblasts recapitulate age-associated disease phenotypes.

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12 December 2020

N6-Furfuryladenine is protective in Huntington's disease models by signaling huntingtin phosphorylation

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12 December 2020

Identifiers

DOI

10.1091/MBC.E18-09-0590

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30 June 2019

PMCID

6249865

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30 June 2019

PubMed ID

30256717

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30 June 2019