CircRNAs belong to a family of RNA molecules which are conserved in evolution, have tissue-specific expression, and are abundant in neuronal cells. Here, we define several features of circ-Hdgfrp3 and describe interesting alterations occurring in motor neurons (MNs) carrying ALS-associated FUS mutations. Through a highly sensitive in situ approach we describe that circ-Hdgfrp3 traffics along neurites, while upon oxidative stress it is retained in the perinuclear region. While in wild-type stressed MNs, circ-Hdgfrp3 localizes in stress granules (SGs), in MNs carrying mutant FUS, a higher proportion of circ-Hdgfrp3 was trapped into cytoplasmic aggregates. Upon stress removal, circ-Hdgfrp3 was easily freed from SGs whereas it was less efficiently released from FUS-aggregates. We found that the human circ-Hdgfrp3 counterpart was also similarly associated to mutant FUS-aggregates in stressed neuronal cells. Overall, the alteration of circ-Hdgfrp3 trafficking adds a further layer of complexity to the role of FUS-aggregates in ALS disease.
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Sydney Med Sch, Sydney, NSW 2006, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Blair, Ian P.
;
Williams, Kelly L.
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Concord Hosp, ANZAC Res Inst, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Williams, Kelly L.
;
Warraich, Sadaf T.
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机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Sydney Med Sch, Sydney, NSW 2006, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Warraich, Sadaf T.
;
Durnall, Jennifer C.
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Concord Hosp, ANZAC Res Inst, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Durnall, Jennifer C.
;
Thoeng, Annora D.
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机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Dept Physiol, Sydney, NSW 2006, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Thoeng, Annora D.
;
Manavis, Jim
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h-index: 0
机构:
Inst Med & Vet Sci, Hanson Inst Ctr Neurol Dis, Adelaide, SA 5000, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Manavis, Jim
;
Blumbergs, Peter C.
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h-index: 0
机构:
Inst Med & Vet Sci, Hanson Inst Ctr Neurol Dis, Adelaide, SA 5000, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Blumbergs, Peter C.
;
Vucic, Steve
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h-index: 0
机构:
Westmead Hosp, Dept Neurol, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Vucic, Steve
;
Kiernan, Matthew C.
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h-index: 0
机构:
Univ New S Wales, Prince Wales Clin Sch, Sydney, NSW, Australia
Prince Wales Med Res Inst, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Kiernan, Matthew C.
;
Nicholson, Garth A.
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h-index: 0
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Sydney Med Sch, Sydney, NSW 2006, Australia
Concord Hosp, Mol Med Lab, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Capauto, Davide
;
Colantoni, Alessio
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h-index: 0
机构:
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Colantoni, Alessio
;
Lu, Lei
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h-index: 0
机构:
Columbia Univ, Dept Neurol, Ctr Motor Neuron Biol & Dis, 630 West 168th St, New York, NY 10032 USAIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Lu, Lei
;
Santini, Tiziana
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Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Santini, Tiziana
;
Peruzzi, Giovanna
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Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Peruzzi, Giovanna
;
Biscarini, Silvia
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h-index: 0
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Biscarini, Silvia
;
Morlando, Mariangela
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h-index: 0
机构:
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Morlando, Mariangela
;
Shneider, Neil A.
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h-index: 0
机构:
Columbia Univ, Dept Neurol, Ctr Motor Neuron Biol & Dis, 630 West 168th St, New York, NY 10032 USAIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Shneider, Neil A.
;
Caffarelli, Elisa
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h-index: 0
机构:
CNR, Inst Mol Biol & Pathol, Piazzale Aldo Moro 7, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Caffarelli, Elisa
;
Laneve, Pietro
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机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
CNR, Inst Mol Biol & Pathol, Piazzale Aldo Moro 7, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
机构:
Univ New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, AustraliaUniv New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
Chen, Bei Jun
;
Huang, Suleigh
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h-index: 0
机构:
Univ New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, AustraliaUniv New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
Huang, Suleigh
;
Janitz, Michael
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h-index: 0
机构:
Univ New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
Univ Leipzig, Paul Flechsig Inst Brain Res, Leipzig, GermanyUniv New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Sydney Med Sch, Sydney, NSW 2006, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Blair, Ian P.
;
Williams, Kelly L.
论文数: 0引用数: 0
h-index: 0
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Williams, Kelly L.
;
Warraich, Sadaf T.
论文数: 0引用数: 0
h-index: 0
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Sydney Med Sch, Sydney, NSW 2006, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Warraich, Sadaf T.
;
Durnall, Jennifer C.
论文数: 0引用数: 0
h-index: 0
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Durnall, Jennifer C.
;
Thoeng, Annora D.
论文数: 0引用数: 0
h-index: 0
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Dept Physiol, Sydney, NSW 2006, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Thoeng, Annora D.
;
Manavis, Jim
论文数: 0引用数: 0
h-index: 0
机构:
Inst Med & Vet Sci, Hanson Inst Ctr Neurol Dis, Adelaide, SA 5000, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Manavis, Jim
;
Blumbergs, Peter C.
论文数: 0引用数: 0
h-index: 0
机构:
Inst Med & Vet Sci, Hanson Inst Ctr Neurol Dis, Adelaide, SA 5000, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Blumbergs, Peter C.
;
Vucic, Steve
论文数: 0引用数: 0
h-index: 0
机构:
Westmead Hosp, Dept Neurol, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Vucic, Steve
;
Kiernan, Matthew C.
论文数: 0引用数: 0
h-index: 0
机构:
Univ New S Wales, Prince Wales Clin Sch, Sydney, NSW, Australia
Prince Wales Med Res Inst, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Kiernan, Matthew C.
;
Nicholson, Garth A.
论文数: 0引用数: 0
h-index: 0
机构:
Concord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
Univ Sydney, Sydney Med Sch, Sydney, NSW 2006, Australia
Concord Hosp, Mol Med Lab, Sydney, NSW, AustraliaConcord Hosp, ANZAC Res Inst, Sydney, NSW, Australia
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Capauto, Davide
;
Colantoni, Alessio
论文数: 0引用数: 0
h-index: 0
机构:
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Colantoni, Alessio
;
Lu, Lei
论文数: 0引用数: 0
h-index: 0
机构:
Columbia Univ, Dept Neurol, Ctr Motor Neuron Biol & Dis, 630 West 168th St, New York, NY 10032 USAIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Lu, Lei
;
Santini, Tiziana
论文数: 0引用数: 0
h-index: 0
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Santini, Tiziana
;
Peruzzi, Giovanna
论文数: 0引用数: 0
h-index: 0
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Peruzzi, Giovanna
;
Biscarini, Silvia
论文数: 0引用数: 0
h-index: 0
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Biscarini, Silvia
;
Morlando, Mariangela
论文数: 0引用数: 0
h-index: 0
机构:
Sapienza Univ Rome, Dept Biol & Biotechnol, Piazzale Aldo Moro 5, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Morlando, Mariangela
;
Shneider, Neil A.
论文数: 0引用数: 0
h-index: 0
机构:
Columbia Univ, Dept Neurol, Ctr Motor Neuron Biol & Dis, 630 West 168th St, New York, NY 10032 USAIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Shneider, Neil A.
;
Caffarelli, Elisa
论文数: 0引用数: 0
h-index: 0
机构:
CNR, Inst Mol Biol & Pathol, Piazzale Aldo Moro 7, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
Caffarelli, Elisa
;
Laneve, Pietro
论文数: 0引用数: 0
h-index: 0
机构:
Ist Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
CNR, Inst Mol Biol & Pathol, Piazzale Aldo Moro 7, I-00185 Rome, ItalyIst Italiano Tecnol, Ctr Life Nano Sci Sapienza, Viale Regina Elena 291, I-00161 Rome, Italy
机构:
Univ New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, AustraliaUniv New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
Chen, Bei Jun
;
Huang, Suleigh
论文数: 0引用数: 0
h-index: 0
机构:
Univ New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, AustraliaUniv New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
Huang, Suleigh
;
Janitz, Michael
论文数: 0引用数: 0
h-index: 0
机构:
Univ New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia
Univ Leipzig, Paul Flechsig Inst Brain Res, Leipzig, GermanyUniv New South Wales, Sch Biotechnol & Biomol Sci, Sydney, NSW 2052, Australia