Evidence map›Paper›PMID 17097067›Full record

ArticleBiochemical and biophysical research communications2006

Proteasome-mediated degradation of integral inner nuclear membrane protein emerin in fibroblasts lacking A-type lamins.

Antoine Muchir, Catherine Massart, Baziel G van Engelen, Martin Lammens, Gisèle Bonne, Howard J Worman

Open access · greenAbstract read
In one paragraph

Article in Biochemical and biophysical research communications, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.

0numbers the graph read from it
0cells of the map it votes in
19citing papers in PubMed
1.5field-weighted citation impact, top 19% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

19 citing papers in PubMed, 37 citations in OpenAlex.

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  19. "Laminopathies": a wide spectrum of human diseases.Experimental cell research · 2007
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 4 institutions in 3 countries.

Antoine MuchirDepartments of Medicine and Anatomy and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA.
Catherine Massart
Baziel G van Engelen
Martin Lammens
Gisèle Bonne
Howard J Worman
Columbia University · USRadboud University Nijmegen · NLFondation pour l’innovation en Cadiométabolisme et Nutrition · FRInstitut de Myologie · FR

Funding

Pathogenesis of Emery-Dreifuss Muscular DystrophyR01AR048997 · NIAMS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI WORMAN, HOWARD J · 2003 to 2024
$7.1M
NIAMS NIH HHS AR048997NIAMS NIH HHS R01 AR048997
6 · The paper itself

Abstract

We previously identified and characterized a homozygous LMNA nonsense mutation leading to the absence of A-type lamins in a premature neonate who died at birth. We show here that the absence of A-type lamins is due to degradation of the aberrant mRNA transcript with a premature termination codon. In cultured fibroblasts from the subject with the homozygous LMNA nonsense mutation, there was a decreased steady-state expression of the integral inner nuclear membrane proteins emerin and nesprin-1alpha associated with their mislocalization to the bulk endoplasmic reticulum and a hyperphosphorylation of emerin. To determine if decreased emerin expression occurred post-translationally, we treated cells with a selective proteasome inhibitor and observed an increase in expression. Our results show that mislocalization of integral inner nuclear membrane proteins to the endoplasmic reticulum in human cells lacking A-type lamins leads to their degradation and provides the first evidence that their degradation is mediated by the proteasome.

Indexed as

Cells, CulturedChildEndoplasmic ReticulumFibroblastsHomozygoteHumansLamin Type AMaleMembrane ProteinsMutationNuclear EnvelopeNuclear ProteinsProteasome Endopeptidase ComplexRNA, MessengerSkinemerinLamin Type AMembrane ProteinsNuclear ProteinsProteasome Endopeptidase ComplexRNA, Messenger

Identifiers

PMID17097067
PMCPMC1771114
OpenAlexW2089681960

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.