Research Article Impairment of chaperone-mediated autophagy

Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney

Festa, Beatrice Paola
Chen, Zhiyong
Berquez, Marine
Debaix, Huguette
Tokonami, Natsuko
Prange, Jenny Ann
Hoek, Glenn Van De
Alessio, Cremonesi
Raimondi, Andrea
Nevo, Nathalie
Giles, Rachel H.
Devuyst, Olivier
Luciani, Alessandro

December 2018

The endolysosomal system sustains the reabsorptive activity of specialized epithelial cells. Lysosom...

A block of autophagy in lysosomal storage disorders

Carmine Settembre
Ro Fraldi
Luca Jahreiss
Carmine Spampanato
Consuelo Venturi
Diego Medina
Raquel De Pablo
Carlo Tacchetti
David C. Rubinsztein
Andrea Ballabio

January 2008

Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal hydrolases. While LS...

A Block of Autophagy in Lysosomal Storage Disorders

Settembre, C
Fraldi, A
Jahreiss, L
Spampanato, C
Venturi, C
MEDINA SANABRIA, Diego Luis
DE PABLO, R
Tacchetti, C
Rubinsztein, Dc
Ballabio, A.

January 2008

Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal hydrolases. While LS...

Research Article Impairment of chaperone-mediated autophagy

Gennaro Napolitano
Jennifer L Johnson
Jing He
Celine J Rocca
Jlenia Monfregola
Kersi Pestonjamasp
Stephanie Cherqui
Sergio D Catz

August 2016

leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosi

Impairment of chaperone‐mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

Napolitano, Gennaro
Johnson, Jennifer L
He, Jing
Rocca, Celine J
Monfregola, Jlenia
Pestonjamasp, Kersi
Cherqui, Stephanie
Catz, Sergio D

February 2015

Metabolite accumulation in lysosomal storage disorders (LSDs) results in impaired cell function and ...

Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

Napolitano, G
Johnson, JL
He, J
Rocca, CJ
Monfregola, J
Pestonjamasp, K
Cherqui, S
Catz, SD

February 2015

Metabolite accumulation in lysosomal storage disorders (LSDs) results in impaired cell function and ...

Defective autophagy degradation and abnormal tight junction-associated signaling drive epithelial dysfunction in cystinosis

Luciani, Alessandro
Festa, Beatrice Paola
Chen, Zhiyong
Devuyst, Olivier

July 2018

Cystinosis is a lysosomal storage disease due to inactivating mutations in CTNS, the cystinosin tran...

Cystinosin, the small GTPase Rab11, and the Rab7 effector RILP regulate intracellular trafficking of the chaperone-mediated autophagy receptor LAMP2A

Zhang, Jinzhong
Johnson, Jennifer L
He, Jing
Napolitano, Gennaro
Ramadass, Mahalakshmi
Rocca, Celine
Kiosses, William B
Bucci, Cecilia
Xin, Qisheng
Gavathiotis, Evripidis
Cuervo, Ana María
Cherqui, Stephanie
Catz, Sergio D

June 2017

The lysosomal storage disease cystinosis, caused by cystinosin deficiency, is characterized by cell ...

Impact of cystinosin glycosylation on protein stability by differential dynamic SILAC

Nevo, Nathalie
Thomas, Lucie
Chhuon, Cerina
Andrzejewska, Zuzanna
Lipecka, Joanna
Guillonneau, François
Bailleux, Anne
Edelman, Aleksander
Antignac, Corinne
Guerrera, Ida Chiara

January 2017

International audienceCystinosis is a rare autosomal recessive lysosomal storage disorder characteri...

Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney

Festa, Beatrice Paola
Chen, Zhiyong
Berquez, Marine
Debaix, Huguette
Tokonami, Natsuko
Prange, Jenny Ann
Hoek, Glenn Van De
Alessio, Cremonesi
Raimondi, Andrea
Nevo, Nathalie
Giles, Rachel H.
Devuyst, Olivier
Luciani, Alessandro

December 2018

The endolysosomal system sustains the reabsorptive activity of specialized epithelial cells. Lysosom...

A block of autophagy in lysosomal storage disorders

Carmine Settembre
Ro Fraldi
Luca Jahreiss
Carmine Spampanato
Consuelo Venturi
Diego Medina
Raquel De Pablo
Carlo Tacchetti
David C. Rubinsztein
Andrea Ballabio

January 2008

Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal hydrolases. While LS...

A Block of Autophagy in Lysosomal Storage Disorders

Settembre, C
Fraldi, A
Jahreiss, L
Spampanato, C
Venturi, C
MEDINA SANABRIA, Diego Luis
DE PABLO, R
Tacchetti, C
Rubinsztein, Dc
Ballabio, A.

January 2008

Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal hydrolases. While LS...

Research Article Impairment of chaperone-mediated autophagy

Gennaro Napolitano
Jennifer L Johnson
Jing He
Celine J Rocca
Jlenia Monfregola
Kersi Pestonjamasp
Stephanie Cherqui
Sergio D Catz

August 2016

leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosi

Impairment of chaperone‐mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

Napolitano, Gennaro
Johnson, Jennifer L
He, Jing
Rocca, Celine J
Monfregola, Jlenia
Pestonjamasp, Kersi
Cherqui, Stephanie
Catz, Sergio D

February 2015

Metabolite accumulation in lysosomal storage disorders (LSDs) results in impaired cell function and ...

Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

Napolitano, G
Johnson, JL
He, J
Rocca, CJ
Monfregola, J
Pestonjamasp, K
Cherqui, S
Catz, SD

February 2015

Metabolite accumulation in lysosomal storage disorders (LSDs) results in impaired cell function and ...

Defective autophagy degradation and abnormal tight junction-associated signaling drive epithelial dysfunction in cystinosis

Luciani, Alessandro
Festa, Beatrice Paola
Chen, Zhiyong
Devuyst, Olivier

July 2018

Cystinosis is a lysosomal storage disease due to inactivating mutations in CTNS, the cystinosin tran...

Cystinosin, the small GTPase Rab11, and the Rab7 effector RILP regulate intracellular trafficking of the chaperone-mediated autophagy receptor LAMP2A

Zhang, Jinzhong
Johnson, Jennifer L
He, Jing
Napolitano, Gennaro
Ramadass, Mahalakshmi
Rocca, Celine
Kiosses, William B
Bucci, Cecilia
Xin, Qisheng
Gavathiotis, Evripidis
Cuervo, Ana María
Cherqui, Stephanie
Catz, Sergio D

June 2017

The lysosomal storage disease cystinosis, caused by cystinosin deficiency, is characterized by cell ...

Impact of cystinosin glycosylation on protein stability by differential dynamic SILAC

Nevo, Nathalie
Thomas, Lucie
Chhuon, Cerina
Andrzejewska, Zuzanna
Lipecka, Joanna
Guillonneau, François
Bailleux, Anne
Edelman, Aleksander
Antignac, Corinne
Guerrera, Ida Chiara

January 2017

International audienceCystinosis is a rare autosomal recessive lysosomal storage disorder characteri...

Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney

Festa, Beatrice Paola
Chen, Zhiyong
Berquez, Marine
Debaix, Huguette
Tokonami, Natsuko
Prange, Jenny Ann
Hoek, Glenn Van De
Alessio, Cremonesi
Raimondi, Andrea
Nevo, Nathalie
Giles, Rachel H.
Devuyst, Olivier
Luciani, Alessandro

December 2018

The endolysosomal system sustains the reabsorptive activity of specialized epithelial cells. Lysosom...

A block of autophagy in lysosomal storage disorders

Carmine Settembre
Ro Fraldi
Luca Jahreiss
Carmine Spampanato
Consuelo Venturi
Diego Medina
Raquel De Pablo
Carlo Tacchetti
David C. Rubinsztein
Andrea Ballabio

January 2008

Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal hydrolases. While LS...

A Block of Autophagy in Lysosomal Storage Disorders

Settembre, C
Fraldi, A
Jahreiss, L
Spampanato, C
Venturi, C
MEDINA SANABRIA, Diego Luis
DE PABLO, R
Tacchetti, C
Rubinsztein, Dc
Ballabio, A.

January 2008

Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal hydrolases. While LS...

Research Article Impairment of chaperone-mediated autophagy

Abstract

Extracted data

Research Article Impairment of chaperone-mediated autophagy

Abstract

Extracted data

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