(Q54447316)

29 May 2018

title

Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex. (English)

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29 May 2018

author

Erich Wanker

10

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29 May 2018

Maciej Lalowski

Maciej Lalowski

9

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Nadine Griesche

2

Nadine Griesche

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29 May 2018

Changwei Chen

4

Changwei Chen

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29 May 2018

Stephanie Dorn

7

Stephanie Dorn

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29 May 2018

author name string

Sybille Krauss

1

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29 May 2018

Ewa Jastrzebska

3

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29 May 2018

Désiree Rutschow

5

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29 May 2018

Clemens Achmüller

6

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29 May 2018

Sylvia M Boesch

8

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29 May 2018

Rainer Schneider

11

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29 May 2018

Susann Schweiger

12

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29 May 2018

language of work or name

English

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publication date

1 January 2013

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29 May 2018

published in

Nature Communications

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29 May 2018

volume

4

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29 May 2018

page(s)

1511

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29 May 2018

Mechanisms of neurodegeneration in Huntington's disease.

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Protein phosphatase 2A (PP2A)-specific ubiquitin ligase MID1 is a sequence-dependent regulator of translation efficiency controlling 3-phosphoinositide-dependent protein kinase-1 (PDPK-1).

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MID1, mutated in Opitz syndrome, encodes an ubiquitin ligase that targets phosphatase 2A for degradation

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mTOR and S6K1 mediate assembly of the translation preinitiation complex through dynamic protein interchange and ordered phosphorylation events

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Muscleblind-like 1 interacts with RNA hairpins in splicing target and pathogenic RNAs

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Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy

21 January 2018

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CAG repeats mimic CUG repeats in the misregulation of alternative splicing

21 January 2018

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Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

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Global changes to the ubiquitin system in Huntington's disease.

21 January 2018

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Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments

21 January 2018

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Induction of autophagy with catalytic mTOR inhibitors reduces huntingtin aggregates in a neuronal cell model.

21 January 2018

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

21 January 2018

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A small-molecule scaffold induces autophagy in primary neurons and protects against toxicity in a Huntington disease model.

21 January 2018

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Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models

21 January 2018

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21 January 2018

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12 December 2020

How does the Huntington's disease mutation damage cells?

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12 December 2020

CAG trinucleotide RNA repeats interact with RNA-binding proteins

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12 December 2020

CAG repeats containing CAA interruptions form branched hairpin structures in spinocerebellar ataxia type 2 transcripts

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Huntingtin processing in pathogenesis of Huntington disease.

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Imperfect CAG repeats form diverse structures in SCA1 transcripts

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Identifiers

DOI

10.1038/NCOMMS2514

1 reference

29 May 2018

ADS bibcode

2013NatCo...4.1511K

0 references

1 reference

29 May 2018