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To aggregate or not to aggregate – Is it a matter of the ribosome?

BioEssays, 2023-07, Vol.45 (7), p.e2200230-n/a

Iben, Sebastian

2023

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Collisions and protein aggregations ahead: how aging affects ribosomal elongation dynamics

Signal transduction and targeted therapy, 2022-04, Vol.7 (1), p.133-133, Article 133

2022

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General loss of proteostasis links Huntington disease to Cockayne syndrome

Neurobiology of disease, 2024-10, Vol.201, p.106668, Article 106668

2024

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Loss of Proteostasis Is a Pathomechanism in Cockayne Syndrome

Cell reports (Cambridge), 2018-05, Vol.23 (6), p.1612-1619

2018

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Cilia defects upon loss of WDR4 are linked to proteasomal hyperactivity and ubiquitin shortage

Cell death & disease, 2024-09, Vol.15 (9), p.660-14, Article 660

2024

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Cockayne Syndrome-Associated CSA and CSB Mutations Impair Ribosome Biogenesis, Ribosomal Protein Stability, and Global Protein Folding

Cells (Basel, Switzerland), 2021-06, Vol.10 (7), p.1616

2021

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Intranuclear inclusions of polyQ-expanded ATXN1 sequester RNA molecules

Frontiers in molecular neuroscience, 2023-12, Vol.16, p.1280546-1280546

2023

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Nucleolar and Ribosomal Dysfunction-A Common Pathomechanism in Childhood Progerias?

Cells (Basel, Switzerland), 2019-06, Vol.8 (6), p.534

2019

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Nuclear actin and myosin I are required for RNA polymerase I transcription

Nature cell biology, 2004-12, Vol.6 (12), p.1165-1172

2004

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Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease

Scientific reports, 2017-08, Vol.7 (1), p.8513-10, Article 8513

2017

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Cellular sensitivity to UV-irradiation is mediated by RNA polymerase I transcription

PloS one, 2017-06, Vol.12 (6), p.e0179843-e0179843

2017

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Ribosomal Dysfunction Is a Common Pathomechanism in Different Forms of Trichothiodystrophy

Cells (Basel, Switzerland), 2023-07, Vol.12 (14), p.1877

2023

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Truncated Cockayne Syndrome B Protein Represses Elongation by RNA Polymerase I

Journal of molecular biology, 2008-10, Vol.382 (2), p.266-274

2008

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TFIIH Plays an Essential Role in RNA Polymerase I Transcription

Cell, 2002-05, Vol.109 (3), p.297-306

2002

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Antigen-Specific Induction of Osteopontin Contributes to the Chronification of Allergic Contact Dermatitis

The American journal of pathology, 2010, Vol.176 (1), p.246-258

2010

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Nucleolar TFIIE plays a role in ribosomal biogenesis and performance

Nucleic acids research, 2021-11, Vol.49 (19), p.11197-11210

2021

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TFIIH mutations can impact on translational fidelity of the ribosome

Human molecular genetics, 2023-03, Vol.32 (7), p.1102-1113

2023

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Ein neues Forum für seltene Hauterkrankungen

Journal der Deutschen Dermatologischen Gesellschaft, 2019-06, Vol.17 (6), p.672-673

2019

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Cockayne syndrome group A and ferrochelatase finely tune ribosomal gene transcription and its response to UV irradiation

Nucleic acids research, 2021-11, Vol.49 (19), p.10911-10930

2021

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A Comparative Assessment of Replication Stress Markers in the Context of Telomerase

Cancers, 2022-04, Vol.14 (9), p.2205

2022

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TFIIH is an elongation factor of RNA polymerase I

Nucleic acids research, 2012-01, Vol.40 (2), p.650-659

2012

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Cockayne syndrome protein A is a transcription factor of RNA polymerase I and stimulates ribosomal biogenesis and growth

Cell cycle (Georgetown, Tex.), 2014-07, Vol.13 (13), p.2029-2037

2014

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806 A circulus vitiosus causes loss of proteostasis in Cockayne syndrome

Journal of investigative dermatology, 2018-05, Vol.138 (5), p.S137-S137

2018

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Analysis of native WT1 protein from frozen human kidney and Wilms' tumors

Oncogene, 1999-04, Vol.18 (15), p.2533-2536

1999

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To aggregate or not to aggregate – Is it a matter of the ribosome?

Collisions and protein aggregations ahead: how aging affects ribosomal elongation dynamics

General loss of proteostasis links Huntington disease to Cockayne syndrome

Loss of Proteostasis Is a Pathomechanism in Cockayne Syndrome

Cilia defects upon loss of WDR4 are linked to proteasomal hyperactivity and ubiquitin shortage

Cockayne Syndrome-Associated CSA and CSB Mutations Impair Ribosome Biogenesis, Ribosomal Protein Stability, and Global Protein Folding

Intranuclear inclusions of polyQ-expanded ATXN1 sequester RNA molecules

Nucleolar and Ribosomal Dysfunction-A Common Pathomechanism in Childhood Progerias?

Nuclear actin and myosin I are required for RNA polymerase I transcription

Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease

Cellular sensitivity to UV-irradiation is mediated by RNA polymerase I transcription

Ribosomal Dysfunction Is a Common Pathomechanism in Different Forms of Trichothiodystrophy

Truncated Cockayne Syndrome B Protein Represses Elongation by RNA Polymerase I

TFIIH Plays an Essential Role in RNA Polymerase I Transcription

Antigen-Specific Induction of Osteopontin Contributes to the Chronification of Allergic Contact Dermatitis

Nucleolar TFIIE plays a role in ribosomal biogenesis and performance

TFIIH mutations can impact on translational fidelity of the ribosome

Ein neues Forum für seltene Hauterkrankungen

Cockayne syndrome group A and ferrochelatase finely tune ribosomal gene transcription and its response to UV irradiation

A Comparative Assessment of Replication Stress Markers in the Context of Telomerase

TFIIH is an elongation factor of RNA polymerase I

Cockayne syndrome protein A is a transcription factor of RNA polymerase I and stimulates ribosomal biogenesis and growth

806 A circulus vitiosus causes loss of proteostasis in Cockayne syndrome

MicroRNA-708 is a novel regulator of the Hoxa9 program in myeloid cells

Analysis of native WT1 protein from frozen human kidney and Wilms' tumors

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