ReviewThe Journal of neuroscience : the official journal of the Society for Neuroscience1998
Deficiency in protein L-isoaspartyl methyltransferase results in a fatal progressive epilepsy.
Review in The Journal of neuroscience : the official journal of the Society for Neuroscience, 1998. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 56 papers.
What it found
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Who cites it
56 citing papers in PubMed, 153 citations in OpenAlex.
- Improved red blood cell storage quality of blood from donors carrying the hypermorphic PIMT I120 variant.HemaSphere · 2026Article
- Untargeted Discovery and Localization of Isomerized Residues in Neuropeptides.Analytical chemistry · 2025Article
- Isomerized Aβ in the brain can distinguish the status of amyloidosis in the Alzheimer's disease spectrum.Acta neuropathologica · 2025Article
- Immunotherapeutic targeting of aging-associated isoDGR motif in chronic lung inflammation.Aging cell · 2025Article
- Isoaspartate-containing galanin in rat hypothalamus.Communications chemistry · 2025Article
- Genesis and regulation of C-terminal cyclic imides from protein damage.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Immunotherapy targeting isoDGR-protein damage extends lifespan in a mouse model of protein deamidation.EMBO molecular medicine · 2023Article
- Biological effects of the loss of homochirality in a multicellular organism.Nature communications · 2022Article
- LC-MS Reveals Isomeric Inhibition of Proteolysis by Lysosomal Cathepsins.Analysis & sensing · 2022Article
- PIMT-Mediated Labeling of l-Isoaspartic Acid with Tris Facilitates Identification of Isomerization Sites in Long-Lived Proteins.Journal of the American Society for Mass Spectrometry · 2022Article
- Protein-L-isoaspartate O-methyltransferase is required forHaematologica · 2021Article
- Isomerization of Asp is essential for assembly of amyloid-like fibrils of αA-crystallin-derived peptide.PloS one · 2021Article
- Quantification of N-terminal amyloid-β isoforms reveals isomers are the most abundant form of the amyloid-β peptide in sporadic Alzheimer's disease.Brain communications · 2021Article
- PIMT Binding to C-Terminal Ala459 of CAIX Is Involved in Inside-Out Signaling Necessary for Its Catalytic Activity.International journal of molecular sciences · 2020Article
- An Unusually Rapid Protein Backbone Modification Stabilizes the Essential Bacterial Enzyme MurA.Biochemistry · 2020Article
- l-Isoaspartyl Methyltransferase Deficiency in Zebrafish Leads to Impaired Calcium Signaling in the Brain.Frontiers in genetics · 2020Article
- Molecular Processes Implicated in Human Age-Related Nuclear Cataract.Investigative ophthalmology & visual science · 2019Review
- Spontaneous Isomerization of Long-Lived Proteins Provides a Molecular Mechanism for the Lysosomal Failure Observed in Alzheimer's Disease.ACS central science · 2019Article
- Protect, repair, destroy or sacrifice: a role of oxidative stress biology in inter-donor variability of blood storage?Blood transfusion = Trasfusione del sangue · 2019Review
- Structural and functional consequences of age-related isomerization in α-crystallins.The Journal of biological chemistry · 2019Article
Corrections and comments
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Authors and funding
18 authors at 8 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Protein L-isoaspartyl methyltransferase (PIMT) is suggested to play a role in the repair of aged protein spontaneously incorporated with isoaspartyl residues. We generated PIMT-deficient mice by targeted disruption of the PIMT gene to elucidate the biological role of the gene in vivo. PIMT-deficient mice died from progressive epileptic seizures with grand mal and myoclonus between 4 and 12 weeks of age. An anticonvulsive drug, dipropylacetic acid (DPA), improved their survival but failed to cure the fatal outcome. L-Isoaspartatate, the putative substrate for PIMT, was increased ninefold in the brains of PIMT-deficient mice. The brains of PIMT-deficient mice started to enlarge after 4 weeks of age when the apical dendrites of pyramidal neurons in cerebral cortices showed aberrant arborizations with disorganized microtubules. We conclude that methylation of modified proteins with isoaspartyl residues is essential for the maintenance of a mature CNS and that a deficiency in PIMT results in fatal progressive epilepsy in mice.
Indexed as
Identifiers
9482793PMC6792936W1511355458What OpenQuestion holds
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.