List of works - Renate K Hukema

An Integrative Study of Protein-RNA Condensates Identifies Scaffolding RNAs and Reveals Players in Fragile X-Associated Tremor/Ataxia Syndrome

scientific article published on 01 December 2018

Antagonistic sensory cues generate gustatory plasticity in Caenorhabditis elegans

scientific article

Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

scientific article published on 26 February 2019

BC RNA Mislocalization in the Fragile X Premutation.

scientific article

CGG-repeat length and neuropathological and molecular correlates in a mouse model for fragile X-associated tremor/ataxia syndrome.

scientific article published on 10 November 2008

Cerebral protein synthesis in a knockin mouse model of the fragile X premutation

scientific article

Combination Therapy in Fragile X Syndrome; Possibilities and Pitfalls Illustrated by Targeting the mGluR5 and GABA Pathway Simultaneously.

scientific article

FMRpolyG-positive inclusions in CNS and non-CNS organs of a fragile X premutation carrier with fragile X-associated tremor/ataxia syndrome

scientific article

Fragile X-associated tremor/ataxia syndrome: Regional decrease of mitochondrial DNA copy number relates to clinical manifestations

scientific article published on 24 May 2019

Gustatory plasticity in C. elegans involves integration of negative cues and NaCl taste mediated by serotonin, dopamine, and glutamate.

scientific article

Induced expression of expanded CGG RNA causes mitochondrial dysfunction in vivo

scientific article

Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome

scientific article

PRKAR1B mutation associated with a new neurodegenerative disorder with unique pathology

scientific article (publication date: May 2014)

Paradoxical effect of baclofen on social behavior in the fragile X syndrome mouse model.

scientific article

Potential pathogenic mechanisms underlying Fragile X Tremor Ataxia Syndrome: RAN translation and/or RNA gain-of-function?

scientific article published on 6 December 2017

Presence of inclusions positive for polyglycine containing protein, FMRpolyG, indicates that repeat-associated non-AUG translation plays a role in fragile X-associated primary ovarian insufficiency

scientific article

RETRACTED: A new inducible transgenic mouse model for C9orf72-associated GGGGCC repeat expansion supports a gain-of-function mechanism in C9orf72-associated ALS and FTD.

scientific article published on 13 December 2014

Reversibility of neuropathology and motor deficits in an inducible mouse model for FXTAS.

scientific article

Selective rescue of heightened anxiety but not gait ataxia in a premutation 90CGG mouse model of Fragile X-associated tremor/ataxia syndrome.

scientific article

Short antisense oligonucleotides alleviate the pleiotropic toxicity of RNA harboring expanded CGG repeats

scientific article published on 24 February 2021

Signaling proteins that regulate NaCl [corrected] chemotaxis responses modulate longevity in C. elegans

scientific article published on 01 July 2009

Small molecule 1a reduces FMRpolyG-mediated toxicity in in vitro and in vivo models for FMR1 premutation

scientific article published on 01 August 2021

The 3' UTR of FMR1 mRNA is a target of miR-101, miR-129-5p and miR-221: implications for the molecular pathology of FXTAS at the synapse

scientific article published on 5 February 2013

The CGG repeat and the FMR1 gene.

scientific article published in January 2013

The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions.

scientific article published on February 2012

The quest for targeted therapy in fragile X syndrome.

scientific article published on 21 August 2015

Translation of Expanded CGG Repeats into FMRpolyG Is Pathogenic and May Contribute to Fragile X Tremor Ataxia Syndrome.

scientific article published on 04 January 2017

Use of model systems to understand the etiology of fragile X-associated primary ovarian insufficiency (FXPOI)

scientific article

Widespread non-central nervous system organ pathology in fragile X premutation carriers with fragile X-associated tremor/ataxia syndrome and CGG knock-in mice ⬇️

scientific article published on July 23, 2011

List of works by Renate K Hukema

An Integrative Study of Protein-RNA Condensates Identifies Scaffolding RNAs and Reveals Players in Fragile X-Associated Tremor/Ataxia Syndrome

Antagonistic sensory cues generate gustatory plasticity in Caenorhabditis elegans

Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

BC RNA Mislocalization in the Fragile X Premutation.

CGG-repeat length and neuropathological and molecular correlates in a mouse model for fragile X-associated tremor/ataxia syndrome.

Cerebral protein synthesis in a knockin mouse model of the fragile X premutation

Combination Therapy in Fragile X Syndrome; Possibilities and Pitfalls Illustrated by Targeting the mGluR5 and GABA Pathway Simultaneously.

FMRpolyG-positive inclusions in CNS and non-CNS organs of a fragile X premutation carrier with fragile X-associated tremor/ataxia syndrome

Fragile X-associated tremor/ataxia syndrome: Regional decrease of mitochondrial DNA copy number relates to clinical manifestations

Gustatory plasticity in C. elegans involves integration of negative cues and NaCl taste mediated by serotonin, dopamine, and glutamate.

Induced expression of expanded CGG RNA causes mitochondrial dysfunction in vivo

Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome

PRKAR1B mutation associated with a new neurodegenerative disorder with unique pathology

Paradoxical effect of baclofen on social behavior in the fragile X syndrome mouse model.

Potential pathogenic mechanisms underlying Fragile X Tremor Ataxia Syndrome: RAN translation and/or RNA gain-of-function?

Presence of inclusions positive for polyglycine containing protein, FMRpolyG, indicates that repeat-associated non-AUG translation plays a role in fragile X-associated primary ovarian insufficiency

RETRACTED: A new inducible transgenic mouse model for C9orf72-associated GGGGCC repeat expansion supports a gain-of-function mechanism in C9orf72-associated ALS and FTD.

Reversibility of neuropathology and motor deficits in an inducible mouse model for FXTAS.

Selective rescue of heightened anxiety but not gait ataxia in a premutation 90CGG mouse model of Fragile X-associated tremor/ataxia syndrome.

Short antisense oligonucleotides alleviate the pleiotropic toxicity of RNA harboring expanded CGG repeats

Signaling proteins that regulate NaCl [corrected] chemotaxis responses modulate longevity in C. elegans

Small molecule 1a reduces FMRpolyG-mediated toxicity in in vitro and in vivo models for FMR1 premutation

The 3' UTR of FMR1 mRNA is a target of miR-101, miR-129-5p and miR-221: implications for the molecular pathology of FXTAS at the synapse

The CGG repeat and the FMR1 gene.

The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions.

The quest for targeted therapy in fragile X syndrome.

Translation of Expanded CGG Repeats into FMRpolyG Is Pathogenic and May Contribute to Fragile X Tremor Ataxia Syndrome.

Use of model systems to understand the etiology of fragile X-associated primary ovarian insufficiency (FXPOI)

Widespread non-central nervous system organ pathology in fragile X premutation carriers with fragile X-associated tremor/ataxia syndrome and CGG knock-in mice ⬇️