ArticleProceedings of the National Academy of Sciences of the United States of America2009
MSC p43 required for axonal development in motor neurons.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
What it found
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Who cites it
26 citing papers in PubMed, 46 citations in OpenAlex.
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- Whole-exome sequencing reveals novel and previously reported variants in genes linked to white matter pathology in neurodevelopmental disorders.European journal of human genetics : EJHG · 2026Article
- Response to Antiangiogenic Therapy Is Associated with AIMP Protein Family Expression in Glioblastoma and Lower-Grade Gliomas.Cancer research communications · 2025Article
- AIMP1: multifunctional regulator in physiology and pathology with therapeutic implications.PeerJ · 2025Review
- Review
- Aminoacyl transfer ribonucleic acid synthetase complex-interacting multifunctional protein 1 induces microglial activation and M1 polarizationFrontiers in cellular neuroscience · 2022Article
- Knockdown of Arginyl-tRNA Synthetase Attenuates Ischemia-Induced Cerebral Cortex Injury in Rats After Middle Cerebral Artery Occlusion.Translational stroke research · 2021Article
- Changes in the expression of endothelial monocyte‑activating polypeptide II in the rat hippocampus following status epilepticus.International journal of molecular medicine · 2021Article
- Roles of aminoacyl-tRNA synthetase-interacting multi-functional proteins in physiology and cancer.Cell death & disease · 2020Review
- Aminoacyl-tRNA synthetases as therapeutic targets.Nature reviews. Drug discovery · 2019Review
- Pathogenic variants in AIMP1 cause pontocerebellar hypoplasia.Neurogenetics · 2019Article
- Article
- Multidirectional inhibition of cortico-hippocampal neurodegeneration by kolaviron treatment in rats.Metabolic brain disease · 2017Article
- Neurofilaments and Neurofilament Proteins in Health and Disease.Cold Spring Harbor perspectives in biology · 2017Review
- Large Conformational Changes of Insertion 3 in Human Glycyl-tRNA Synthetase (hGlyRS) during Catalysis.The Journal of biological chemistry · 2016Article
- Missense variants in AIMP1 gene are implicated in autosomal recessive intellectual disability without neurodegeneration.European journal of human genetics : EJHG · 2016Article
- Identification of a Vav2-dependent mechanism for GDNF/Ret control of mesolimbic DAT trafficking.Nature neuroscience · 2015Article
- The N terminus of pro-endothelial monocyte-activating polypeptide II (EMAP II) regulates its binding with the C terminus, arginyl-tRNA synthetase, and neurofilament light protein.The Journal of biological chemistry · 2015Article
- Structure of the ArgRS-GlnRS-AIMP1 complex and its implications for mammalian translation.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
- AIMP1 deficiency presents as a cortical neurodegenerative disease with infantile onset.Neurogenetics · 2014Article
Corrections and comments
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Authors and funding
7 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Neuron connectivity and correct neural function largely depend on axonal integrity. Neurofilaments (NFs) constitute the main cytoskeletal network maintaining the structural integrity of neurons and exhibit dynamic changes during axonal and dendritic growth. However, the mechanisms underlying axonal development and maintenance remain poorly understood. Here, we identify that multisynthetase complex p43 (MSC p43) is essential for NF assembly and axon maintenance. The MSC p43 protein was predominantly expressed in central neurons and interacted with NF light subunit in vivo. Mice lacking MSC p43 exhibited axon degeneration in motor neurons, defective neuromuscular junctions, muscular atrophy, and motor dysfunction. Furthermore, MSC p43 depletion in mice caused disorganization of the axonal NF network. Mechanistically, MSC p43 is required for maintaining normal phosphorylation levels of NFs. Thus, MSC p43 is indispensable in maintaining axonal integrity. Its dysfunction may underlie the NF disorganization and axon degeneration associated with motor neuron degenerative diseases.
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Registered trials
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.