Early autophagic response in a novel knock-in model of Huntington disease (Q34112214)

31 July 2017

Early autophagic response in a novel knock-in model of Huntington disease (English)

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31 July 2017

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Mary Y Heng

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31 July 2017

Duy K Duong

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31 July 2017

Roger L Albin

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31 July 2017

Sara J Tallaksen-Greene

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31 July 2017

Jesse M Hunter

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Mathieu J Lesort

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31 July 2017

Alex Osmand

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31 July 2017

Henry L Paulson

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31 July 2017

Peter J Detloff

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31 July 2017

8 July 2010

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31 July 2017

Human Molecular Genetics

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31 July 2017

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31 July 2017

3702-3720

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describes a project that uses

31 July 2017

ImageJ

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC2935855/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

cites work

Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease

1 reference

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The selective autophagy substrate p62 activates the stress responsive transcription factor Nrf2 through inactivation of Keap1

5 July 2018

1 reference

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

5 July 2018

1 reference

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IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3

5 July 2018

1 reference

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Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment

5 July 2018

1 reference

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Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.

5 July 2018

1 reference

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Inhibition of autophagy induction delays neuronal cell loss caused by dysfunctional ESCRT-III in frontotemporal dementia

5 July 2018

1 reference

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Insulin-like growth factor-I prevents the accumulation of autophagic vesicles and cell death in Purkinje neurons by increasing the rate of autophagosome-to-lysosome fusion and degradation

5 July 2018

1 reference

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

5 July 2018

1 reference

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Mouse models of Huntington's disease and methodological considerations for therapeutic trials

5 July 2018

1 reference

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Acetylation targets mutant huntingtin to autophagosomes for degradation

5 July 2018

1 reference

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Autophagy regulates lipid metabolism

5 July 2018

1 reference

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Cytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy

5 July 2018

1 reference

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CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse

5 July 2018

1 reference

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Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Polyglutamine neurodegeneration: protein misfolding revisited.

5 July 2018

1 reference

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Autophagy in neurodegeneration and development

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Rodent genetic models of Huntington disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Huntington's disease: degradation of mutant huntingtin by autophagy

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Autophagy induction and autophagosome clearance in neurons: relationship to autophagic pathology in Alzheimer's disease

5 July 2018

1 reference

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Atg8 controls phagophore expansion during autophagosome formation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Huntington's disease: from pathology and genetics to potential therapies

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Regulation of autophagy by cytoplasmic p53

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Premature aging in mice activates a systemic metabolic response involving autophagy induction.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Autophagosome formation: core machinery and adaptations

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington's disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

How to interpret LC3 immunoblotting

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Imaging polyglutamine deposits in brain tissue

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Loss of autophagy in the central nervous system causes neurodegeneration in mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Human light chain 3/MAP1LC3B is cleaved at its carboxyl-terminal Met121 to expose Gly120 for lipidation and targeting to autophagosomal membranes

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Autophagosome-like vacuole formation in Huntington's disease lymphoblasts

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Alzheimer's disease is a synaptic failure

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Impairment of the ubiquitin-proteasome system by protein aggregation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Reversing neurodegeneration: a promise unfolds

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Are there multiple pathways in the pathogenesis of Huntington's disease?

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Huntingtin localization in brains of normal and Huntington's disease patients

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

5 July 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Acute polyglutamine expression in inducible mouse model unravels ubiquitin/proteasome system impairment and permanent recovery attributable to aggregate formation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Trehalose, a novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Novel targets for Huntington's disease in an mTOR-independent autophagy pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Behavioral abnormalities precede neuropathological markers in rats transgenic for Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

A comparison of huntington disease and huntington disease-like 2 neuropathology

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

In search of an "autophagomometer".

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Autophagy regulates the processing of amino terminal huntingtin fragments

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Monitoring autophagic degradation of p62/SQSTM1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDQ285

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Mouse Huntington's disease homolog mRNA levels: variation and allele effects

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations.

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2935855

28 October 2018

Identifiers

DOI

10.1093/HMG/DDQ285

1 reference

31 July 2017

1 reference

31 July 2017

1 reference