Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Regulation of SMN protein stability.

Molecular and cellular biology - 1 Mar 2009

Burnett Barrington G, Muñoz Eric, Tandon Animesh, Kwon Deborah Y, Sumner Charlotte J, Fischbeck Kenneth H

Abstract excerpt

Spinal muscular atrophy (SMA) is caused by mutations of the survival of motor neuron (SMN1) gene and deficiency of full-length SMN protein (FL-SMN). All SMA patients retain one or more copies of the SMN2 gene, but the principal protein product of SMN2 lacks exon 7 (SMNDelta7) and is unable to compensate for a deficiency of FL-SMN. SMN is known to oligomerize and form a multimeric protein complex; however, the...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Dimerization
Share to Half-Life
Share to Multiprotein Complexes

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Mutation

Share to Proteasome Endopeptidase Complex

Share to Protein Stability

Share to Survival of Motor Neuron 1 Protein

Share to Ubiquitination

Source

Open in PubMed Open full text

Identifiers

PMID: 19103745
DOI: 10.1128/MCB.01262-08
PMCID: PMC2643817

Genes

SMN2
SMN1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Regulation of SMN protein stability.PMID 19103745Read ↗

Gemin2 plays an important role in stabilizing the survival of motor neuron complex.Semantic proximity Read ↗

Gemins modulate the expression and activity of the SMN complexSemantic proximity Read ↗

The SMN Complex at the Crossroad between RNA Metabolism and NeurodegenerationSemantic proximity Read ↗

SMNΔ7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMNSemantic proximity Read ↗

The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiationSemantic proximity Read ↗

SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.Semantic proximity Read ↗

A Comprehensive Interaction Map of the Human Survival of Motor Neuron (SMN) ComplexSemantic proximity Read ↗

Blocking p62-dependent SMN degradation ameliorates spinal muscular atrophy disease phenotypes.Semantic proximity Read ↗

Gemin5 Binds to the Survival Motor Neuron mRNA to Regulate SMN ExpressionSemantic proximity Read ↗

Spliceosomal small nuclear ribonucleoprotein biogenesis defects and motor neuron selectivity in spinal muscular atrophy.Semantic proximity Read ↗

SMN-independent subunits of the SMN complex. Identification of a small nuclear ribonucleoprotein assembly intermediate.Semantic proximity Read ↗

A novel human-specific splice isoform alters the critical C-terminus of Survival Motor Neuron proteinSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.