Affinage

AGTPBP1

Cytosolic carboxypeptidase 1 · UniProt Q9UPW5

Length
1226 aa
Mass
138.4 kDa
Annotated
2026-04-28
53 papers in source corpus 25 papers cited in narrative 25 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

AGTPBP1 (also known as Nna1/CCP1) is a zinc-dependent metallocarboxypeptidase of the M14D subfamily that removes C-terminal glutamate residues from polyglutamylated tubulin and other substrates, thereby governing microtubule post-translational modification homeostasis across diverse cell types including neurons, photoreceptors, spermatogenic cells, and microglia (PMID:22835831, PMID:30225910, PMID:37937809, PMID:41475674). Loss of its deglutamylase activity causes tubulin hyperglutamylation that impairs microtubule-dependent axonal transport, mitochondrial dynamics, and synaptic receptor trafficking, leading to Purkinje cell, motor neuron, photoreceptor, and spermatogenic cell death, with associated ER stress and unfolded protein response activation (PMID:30337352, PMID:33004692, PMID:30420557, PMID:40989022). Biallelic loss-of-function variants in humans cause infantile-onset neurodegeneration with cerebellar and peripheral nerve involvement, while missense variants in the catalytic domain cause teratozoospermia (PMID:30420557, PMID:37937809, PMID:41160201). An N-terminal nuclear localization signal additionally supports cognition-related synaptic functions—including AMPA receptor trafficking—independent of the carboxypeptidase catalytic domain (PMID:36361749).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 2000 Medium

    Establishing AGTPBP1 as a protein with a predicted carboxypeptidase domain and dual cytoplasmic-nuclear localization in neurons provided the first structural and spatial framework for understanding its function.

    Evidence GFP-fusion protein transfection and fluorescence microscopy in cultured neurons; sequence analysis of zinc carboxypeptidase and ATP/GTP-binding domains

    PMID:11083920

    Open questions at the time
    • Enzymatic activity not demonstrated
    • Nuclear function undefined
    • ATP/GTP binding role unclear
  2. 2002 High

    Positional cloning of the pcd locus revealed that loss-of-function mutations in AGTPBP1 cause degeneration of Purkinje cells, photoreceptors, mitral neurons, and spermatogenic cells, establishing the gene's essentiality for survival of multiple neuronal and germ cell populations.

    Evidence Positional cloning and mutational analysis across three independent pcd alleles in mice

    PMID:11884758

    Open questions at the time
    • Enzymatic substrate unknown
    • Mechanism of cell death unknown
  3. 2006 High

    Demonstrating that the substrate-binding site and zinc-binding domain are each required for in vivo rescue of Purkinje cell degeneration proved that the carboxypeptidase catalytic activity—not merely protein scaffolding—is the essential neuroprotective function.

    Evidence Transgenic and BAC transgenic rescue with site-directed mutations of substrate-binding and zinc-binding residues in pcd mice; behavioral and histological analysis

    PMID:16952463 PMID:18602413

    Open questions at the time
    • Physiological substrate not yet identified biochemically
    • Whether ATP/GTP binding contributes to function in vivo remained untested
  4. 2007 High

    Biochemical reconstitution of a C. elegans ortholog demonstrated that the M14D subfamily possesses carboxypeptidase activity stimulated by ATP/ADP and inhibited by Z-Glu-Tyr, consistent with tubulin C-terminal processing.

    Evidence Recombinant protein expression and in vitro carboxypeptidase assay with synthetic peptides; inhibitor profiling

    PMID:17244817

    Open questions at the time
    • Tubulin not directly tested as substrate
    • Mammalian Nna1 not yet reconstituted
  5. 2010 High

    Two parallel lines of investigation revealed that CCP1 loss causes accumulation of proteasome-generated intracellular peptides with elevated autophagy, and also disrupts NF-κB signaling and microtubule-dependent Purkinje cell dendrite development, broadening the mechanistic framework beyond a single substrate.

    Evidence Quantitative peptidomics and autophagy markers in pcd brain; single-cell gene profiling and viral rescue in pcd Purkinje neurons

    PMID:20061535 PMID:20920790

    Open questions at the time
    • Relative contribution of tubulin deglutamylation vs. peptide turnover to neurodegeneration unresolved
    • Peptide turnover function not reconstituted with purified enzyme
  6. 2012 High

    Purification and enzymatic characterization of recombinant mammalian Nna1 definitively established it as a tubulin deglutamylase that preferentially removes C-terminal glutamates from polyglutamylated substrates, with catalytic mutants validated both in vitro and in vivo, while the ATP/GTP binding site proved dispensable for neuronal rescue.

    Evidence Purified recombinant Nna1 enzymatic assay showing preference for ≥2 glutamate substrates; catalytic mutant transgenic rescue failure in pcd mice; ATP/GTP site mutant rescue success

    PMID:22835831

    Open questions at the time
    • Crystal structure not solved
    • Full repertoire of physiological substrates not defined
  7. 2015 Medium

    Discovery that agtpbp1 is required for T lymphocyte development in zebrafish extended the gene's functional repertoire to the immune system, beyond its established roles in neurons and germ cells.

    Evidence Gene-breaking transposon mutagenesis and morpholino knockdown in zebrafish with flow cytometry

    PMID:26161877

    Open questions at the time
    • Mechanism of T cell development impairment unknown
    • Whether tubulin hyperglutamylation is causative in immune cells not tested
  8. 2018 High

    Three key advances converged: CCP1 loss was shown to impair mitochondrial fusion and axonal transport via tubulin hyperglutamylation (with a Parkin physical interaction identified); conditional carboxypeptidase-domain deletion confirmed ER stress as a cell death mechanism; and biallelic human AGTPBP1 variants were identified as causing infantile-onset neurodegeneration.

    Evidence Drosophila genetic epistasis and live imaging of mitochondrial dynamics; co-IP of CCP1 and Parkin; conditional knockout with ER stress markers; whole-exome sequencing in human patients with functional validation

    PMID:30225910 PMID:30337352 PMID:30420557

    Open questions at the time
    • Parkin-CCP1 interaction not structurally characterized
    • Genotype-phenotype spectrum in human disease not fully defined
    • Whether ER stress is a direct or indirect consequence of hyperglutamylation unclear
  9. 2020 Medium

    Pharmacological microtubule depolymerizer treatment partially rescued the glutamylation imbalance and improved Purkinje neuron survival, directly linking the hyperglutamylated microtubule pool—not just soluble tubulin—to the cell death cascade involving ER stress and apoptosis/necroptosis.

    Evidence Laser capture microdissection of pcd Purkinje neurons; microtubule depolymerizer administration; ER stress and cell death pathway analysis

    PMID:33004692

    Open questions at the time
    • Therapeutic window and long-term efficacy unknown
    • Molecular sensors linking hyperglutamylation to ER stress not identified
  10. 2021 Medium

    Deletion of the N-terminal nuclear localization signal (exon 3) showed that Nna1 has a non-catalytic, NLS-dependent role in synaptic AMPA receptor (GluA2) and kinesin-1 trafficking that supports fear memory and anxiety behavior, dissociating nuclear from cytoplasmic functions.

    Evidence Exon 3 conditional KO mice; behavioral testing; synaptosomal fractionation and western blot for GluA2 and kinesin-1

    PMID:36361749

    Open questions at the time
    • Nuclear binding partners mediating this function unknown
    • Whether NLS-dependent function involves nuclear deglutamylation or a scaffolding role is unresolved
  11. 2023 High

    Localization of AGTPBP1 to the spermatid manchette and identification of human missense variants causing teratozoospermia established tubulin deglutamylation as critical for sperm head and tail morphogenesis.

    Evidence Immunofluorescence in murine spermatids; Agtpbp1-null mouse sperm analysis; whole-exome sequencing in infertile men

    PMID:37937809

    Open questions at the time
    • Manchette-specific substrates beyond tubulin not identified
    • Functional rescue of human variants not performed
  12. 2025 Medium

    Multiple studies expanded CCP1's functional scope: GABAergic neuron-specific KO demonstrated its requirement for hippocampal parvalbumin interneuron integration; microglia were shown to have intrinsic CCP1-dependent motility, phagocytosis, and proliferation deficits; CRMP2 was identified as a downstream effector of AGTPBP1 loss in neurons; and a knock-in mouse confirmed causality of the human R791H variant for sperm defects.

    Evidence Conditional KO in GABAergic neurons with electrophysiology; isolated pcd microglial functional assays; CRISPR KO with lacosamide rescue in neurons and zebrafish; CRISPR knock-in of R791H mutation in mice

    PMID:40347376 PMID:40989022 PMID:41160201 PMID:41475674

    Open questions at the time
    • Whether CRMP2 is a direct deglutamylation substrate of CCP1 is unknown
    • Microglial phenotype contribution to neurodegeneration vs. independent role not resolved
    • How deglutamylation specifically regulates PV interneuron axonal transport remains mechanistically undefined

Open questions

Synthesis pass · forward-looking unresolved questions
  • Major unresolved questions include the full substrate repertoire beyond tubulin, the structural basis for substrate selectivity, whether the peptide turnover and tubulin deglutamylation functions are separable contributors to neurodegeneration, the molecular mechanism by which the NLS-dependent nuclear function regulates synaptic receptor trafficking, and the therapeutic potential of modulating tubulin glutamylation in human CCP1-deficient disease.
  • No crystal or cryo-EM structure of mammalian CCP1 solved
  • Relative contributions of tubulin deglutamylation vs. peptide turnover to neurodegeneration not genetically separated
  • NLS-dependent nuclear interactome uncharacterized

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016787 hydrolase activity 2 GO:0140096 catalytic activity, acting on a protein 2
Localization
GO:0005634 nucleus 2 GO:0005829 cytosol 2 GO:0005856 cytoskeleton 1
Pathway
R-HSA-1852241 Organelle biogenesis and maintenance 2 R-HSA-392499 Metabolism of proteins 2 R-HSA-5357801 Programmed Cell Death 2 R-HSA-9612973 Autophagy 1
Partners
CRMP2PARK2TUBA1ATUBB4B

Evidence

Reading pass · 25 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2002 Loss-of-function mutations in Nna1 (AGTPBP1) cause the Purkinje cell degeneration (pcd) phenotype, including adult-onset degeneration of cerebellar Purkinje neurons, retinal photoreceptors, olfactory bulb mitral neurons, selected thalamic neurons, and defective spermatogenesis, establishing Nna1 as the causative gene. Positional cloning and mutational analysis of three pcd alleles; genetic linkage Science High 11884758
2000 AGTPBP1/Nna1 encodes a protein with a zinc carboxypeptidase domain and ATP/GTP binding motif; GFP-Nna1 fusion protein localizes to both cytoplasmic and nuclear compartments in cultured neurons. GFP fusion protein transfection and fluorescence microscopy; sequence analysis Molecular and cellular neurosciences Medium 11083920
2006 The carboxypeptidase-like substrate-binding site of Nna1 is essential for neuronal survival; transgenic expression of wild-type but not substrate-binding-site mutant Nna1 rescues Purkinje cell loss and ataxic behavior in pcd mice. Transgenic rescue with site-directed mutagenesis of substrate-binding site; behavioral and histological analysis in pcd background Molecular and cellular neurosciences High 16952463
2007 Nna1-like proteins constitute a new M14D subfamily of metallocarboxypeptidases with an unusually open active site; a C. elegans Nna1-like ortholog cleaves C-terminal amino acids from synthetic peptides, with activity stimulated by ATP/ADP, inhibited by Z-Glu-Tyr, and consistent with tubulin carboxypeptidase function. Recombinant protein expression and in vitro carboxypeptidase assay; phylogenetic analysis; conformational modeling FASEB journal High 17244817
2008 The zinc-binding domain of Nna1 is required to prevent retinal photoreceptor loss and cerebellar Purkinje cell degeneration; mutation of the zinc-binding domain in a BAC transgene abolishes rescue of pcd phenotypes. BAC transgenic rescue with zinc-binding domain mutation in pcd mice; histological analysis Vision research High 18602413
2010 Loss of CCP1/Nna1 leads to accumulation of hundreds of intracellular peptides (proteasome-generated) in multiple brain regions, suggesting CCP1 functions in protein turnover by cleaving proteasome-generated peptides into amino acids; reduced amino acid levels trigger elevated autophagy in pcd mouse brain. Quantitative proteomics and peptidomics (2-D gel, mass spectrometry); proteasome activity assay; autophagy markers in pcd vs. wild-type mice FASEB journal High 20061535
2010 Mutant Nna1 (pcd) dramatically increases intranuclear localization of lysyl oxidase propeptide, which interferes with NF-κB RelA signaling and microtubule-associated protein regulation of microtubule stability, leading to underdevelopment of Purkinje cell dendrites. Single-cell gene profiling; virus-based gene transfer rescue; immunofluorescence in pcd mice Neuron Medium 20920790
2010 AGTPBP1 is expressed in spermatogenic cells from late primary spermatocyte to round spermatid stage; its loss in pcd mice causes defective spermatogenesis beginning at the pachytene spermatocyte stage with increased apoptosis, altered expression of cyclin B3, USP2, and USP9y. Immunohistochemistry with anti-AGTPBP1 antibody; histological analysis; global gene expression analysis of pcd testes Molecules and cells Medium 21110128
2012 Recombinant Nna1 is a monomeric metallocarboxypeptidase that removes glutamate from tubulin; it preferentially hydrolyzes substrates with ≥2 C-terminal glutamate residues (Vmax ~7-fold higher for triglutamate vs. diglutamate); catalytic mutants (D1007E, R1078E, E1094A) inactivate Nna1 both in vitro and in vivo; ATP/GTP binding site mutation has no effect on neuronal rescue. Purified recombinant Nna1 in vitro enzymatic assay; site-directed mutagenesis; transgenic rescue in pcd mice FASEB journal High 22835831
2012 A missense mutation (Arg970Pro) in the catalytic domain of AGTPBP1 causes lower motor neuron disease in sheep, demonstrating that catalytic activity of AGTPBP1 is required for motor neuron survival in a large mammal model. Genome-wide homozygosity mapping; Sanger sequencing; genotyping concordance analysis Heredity Medium 22588130
2018 CCP1/Nna1 promotes mitochondrial fusion and motility; loss of CCP1 causes mitochondrial fragmentation in cells and pcd neurons; tubulin hyperglutamylation due to CCP1 loss impairs microtubule-mediated retrograde mitochondrial axonal transport; CCP1 and Parkin physically interact; mitochondrial stress promotes Parkin-dependent turnover of CCP1. Drosophila genetic epistasis (Drp1 dosage modulation); live imaging of mitochondrial dynamics; mitochondrial transport assays in pcd neurons; co-immunoprecipitation of CCP1 and Parkin The Journal of cell biology High 30337352
2018 Loss of CCP1 (AGTPBP1) in humans causes infantile-onset neurodegeneration with dysregulated tubulin polyglutamylation; biallelic variants confirmed absence of functional CCP1 protein and accumulation of hyperglutamylated tubulin in degenerating neurons; pcd mice recapitulate peripheral nerve and spinal motor neuron degeneration. Whole-exome sequencing; functional cDNA studies; immunostaining for polyglutamylated tubulin in patient and mouse tissues The EMBO journal High 30420557
2018 Deletion of the carboxypeptidase domain of Nna1 in conditional knockout mice causes Purkinje cell degeneration with increased polyglutamylated tubulin, endoplasmic reticulum stress (elevated CHOP), and activation of microglia and astrocytes, establishing the carboxypeptidase domain as essential for PC survival and linking ER stress to CCP1 loss. Conditional knockout (Cre-lox deletion of carboxypeptidase domain exons); immunohistochemistry; western blot for polyglutamylated tubulin and ER stress markers Journal of neurochemistry High 30225910
2020 In pcd Purkinje neurons, absence of Nna1 deglutamylase leads to hyperglutamylated microtubules and tubulin dimers; treatment with a microtubule depolymerizer corrects the glutamylation/deglutamylation ratio and increases Purkinje neuron survival; ER stress, unfolded protein response, and protein synthesis inhibition precede Purkinje neuron death by apoptosis/necroptosis. Laser capture microdissection of single Purkinje neurons; immunostaining; pharmacological depolymerizer treatment; analysis of ER stress markers JCI insight Medium 33004692
2021 CCP1 deglutamylase activity protects rodent spinal cord neurons from glutamate-induced excitotoxic death; shRNA-mediated knockdown of CCP1 increases susceptibility to excitotoxicity; excitotoxic injury reduces CCP1 expression; the neuroprotective effect is independent of neuronal cilia maintenance. shRNA knockdown of CCP1 in embryonic rat spinal cord cultures; glutamate excitotoxicity assay; cilia length measurement eNeuro Medium 33688040
2023 AGTPBP1 localizes to the manchette structure in spermatids (essential for sperm head/tail formation); loss of AGTPBP1 (null mice) causes abnormal polyglutamylated tubulin accumulation and decreased Δ-2 tubulin, resulting in sperm head and flagella defects; missense variants in human AGTPBP1 (p.Glu423Asp, p.Pro631Leu, p.Arg811His) cause teratozoospermia. Immunofluorescence localization in murine spermatids; Agtpbp1-null mouse analysis; whole-exome sequencing in infertile men; western blot for polyglutamylated and Δ-2 tubulin Journal of cellular and molecular medicine High 37937809
2015 agtpbp1 is essential for T lymphocyte development in zebrafish; homozygous agtpbp1 mutants exhibit impaired T cell development, and morpholino-mediated knockdown recapitulates the T cell defect. Gene-breaking transposon mutagenesis screen; flow cytometry of immune cells; morpholino knockdown in zebrafish PloS one Medium 26161877
2015 AGTPBP1 co-immunoprecipitates with C9orf72 in transfected cells and co-localizes with C9orf72 in human brain neurons, suggesting a physical interaction between these proteins. Co-immunoprecipitation in transfected cells; double-labeling immunohistochemistry in human brain Journal of central nervous system disease Low 26106267
2025 Conditional knockout of Ccp1 in GABAergic neurons impairs MT-dependent axonal transport and reduces perisomatic inhibition of pyramidal cells by parvalbumin interneurons in the CA2 hippocampal region, demonstrating that tubulin deglutamylation by CCP1 is required for functional integration of hippocampal PV interneurons. Conditional knockout (GABAergic neuron-specific Ccp1 depletion); electrophysiology; axonal transport assays; immunofluorescence iScience Medium 40989022
2024 AGTPBP1 knockdown in pancreatic cancer cells inhibits ERK1/2 phosphorylation, reduces MYLK and TUBB4B protein levels, and suppresses cell proliferation and migration in vitro and in vivo, placing AGTPBP1 upstream of the ERK signaling pathway in cancer cells. siRNA knockdown; western blot; RNA sequencing; in vivo xenograft Molecular medicine Low 39129004
2025 AGTPBP1 knockout leads to excessive neurite outgrowth, increased CRMP2 expression, mitochondrial dysfunction, and a hyperdopaminergic state in differentiated neurons; in zebrafish, AGTPBP1 knockdown reduces brain volume and impairs motor function; lacosamide (CRMP2 modulator) rescues these defects, implicating elevated CRMP2 downstream of AGTPBP1 loss. CRISPR KO cell model; zebrafish morpholino knockdown; pharmacological rescue with lacosamide; mitochondrial function assays Molecular neurobiology Medium 40347376
2025 CCP1 inhibits pulmonary fibrosis by binding to EIF4B and suppressing the PI3K/AKT signaling pathway; CCP1 knockdown promotes fibroblast migration, proliferation, and fibrotic transformation, while overexpression inhibits these processes; co-immunoprecipitation confirmed CCP1-EIF4B interaction. Co-immunoprecipitation; siRNA knockdown and overexpression; western blot; in vivo bleomycin model; RNA-seq Cell biology international Low 40590568
2025 Ccp1-deficient microglia (from pcd mice) exhibit intrinsic deficits in phagocytosis, motility, and proliferation independent of neuronal loss; microglia adopt an anti-inflammatory rather than pro-inflammatory activation profile in degenerating regions, implicating CCP1-dependent cytoskeletal dynamics in microglial homeostasis. In vitro microglial isolation and functional assays (phagocytosis, motility, proliferation); in vivo CSF1R inhibitor depletion; transcriptomic profiling; immunohistochemistry Brain, behavior, and immunity Medium 41475674
2021 Nna1 KO mice lacking the N-terminal nuclear localization signal (exon 3 deletion) do not develop the pcd Purkinje cell degeneration phenotype but show reduced hippocampal GluA2 AMPA receptor and kinesin-1 in the synaptosomal fraction, impaired contextual and auditory fear memory, and altered anxiety behavior, indicating Nna1 has NLS-dependent roles in synaptic receptor trafficking and cognition independent of its carboxypeptidase function. Conditional KO (exon 3 deletion); behavioral testing (elevated plus maze, light/dark box, fear conditioning); biochemical fractionation and western blot for GluA2 and kinesin-1 International journal of molecular sciences Medium 36361749
2025 The human teratozoospermia-associated AGTPBP1 R791H (R811H) mutation causes sperm head and tail morphological defects in knock-in mice, with abnormal accumulation of polyglutamylated tubulin in sperm heads, confirming causality of this mutation in impaired tubulin deglutamylation and sperm differentiation. CRISPR knock-in mouse model; sperm morphological evaluation; immunofluorescence for polyglutamylated tubulin Journal of assisted reproduction and genetics High 41160201

Source papers

Stage 0 corpus · 53 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2002 Purkinje cell degeneration (pcd) phenotypes caused by mutations in the axotomy-induced gene, Nna1. Science (New York, N.Y.) 190 11884758
2003 CCP1-4 of the C4b-binding protein alpha-chain are required for factor I mediated cleavage of complement factor C3b. Molecular immunology 119 12431388
2018 Loss of tubulin deglutamylase CCP1 causes infantile-onset neurodegeneration. The EMBO journal 102 30420557
2007 Nna1-like proteins are active metallocarboxypeptidases of a new and diverse M14 subfamily. FASEB journal : official publication of the Federation of American Societies for Experimental Biology 87 17244817
2000 Regenerating motor neurons express Nna1, a novel ATP/GTP-binding protein related to zinc carboxypeptidases. Molecular and cellular neurosciences 79 11083920
2010 Nna1 mediates Purkinje cell dendritic development via lysyl oxidase propeptide and NF-κB signaling. Neuron 62 20920790
1999 The mitochondrial cytochrome c peroxidase Ccp1 of Saccharomyces cerevisiae is involved in conveying an oxidative stress signal to the transcription factor Pos9 (Skn7). Molecular & general genetics : MGG 59 10589830
2010 CCP1/Nna1 functions in protein turnover in mouse brain: Implications for cell death in Purkinje cell degeneration mice. FASEB journal : official publication of the Federation of American Societies for Experimental Biology 48 20061535
2004 The Chlamydomonas reinhardtii proteins Ccp1 and Ccp2 are required for long-term growth, but are not necessary for efficient photosynthesis, in a low-CO2 environment. Plant molecular biology 45 15604732
2006 The Kaposi's sarcoma-associated herpesvirus complement control protein (KCP) binds to heparin and cell surfaces via positively charged amino acids in CCP1-2. Molecular immunology 43 16442624
2006 The carboxypeptidase-like substrate-binding site in Nna1 is essential for the rescue of the Purkinje cell degeneration (pcd) phenotype. Molecular and cellular neurosciences 42 16952463
1987 A serine protease (CCP1) is sequestered in the cytoplasmic granules of cytotoxic T lymphocytes. Journal of immunology (Baltimore, Md. : 1950) 40 2445811
2008 The zinc-binding domain of Nna1 is required to prevent retinal photoreceptor loss and cerebellar ataxia in Purkinje cell degeneration (pcd) mice. Vision research 31 18602413
2007 Immunophysical exploration of C3d-CR2(CCP1-2) interaction using molecular dynamics and electrostatics. Journal of molecular biology 29 17434528
2006 The Purkinje cell degeneration 5J mutation is a single amino acid insertion that destabilizes Nna1 protein. Mammalian genome : official journal of the International Mammalian Genome Society 28 16465590
2018 CCP1 promotes mitochondrial fusion and motility to prevent Purkinje cell neuron loss in pcd mice. The Journal of cell biology 27 30337352
2019 Biallelic variants in AGTPBP1, involved in tubulin deglutamylation, are associated with cerebellar degeneration and motor neuropathy. European journal of human genetics : EJHG 25 30976113
2018 Deletion of exons encoding carboxypeptidase domain of Nna1 results in Purkinje cell degeneration (pcd) phenotype. Journal of neurochemistry 25 30225910
2000 Positively charged amino acids at the interface between alpha-chain CCP1 and CCP2 of C4BP are required for regulation of the classical C3-convertase. Molecular immunology 25 11090879
2016 Ccp1 Homodimer Mediates Chromatin Integrity by Antagonizing CENP-A Loading. Molecular cell 22 27666591
2012 A missense mutation in AGTPBP1 was identified in sheep with a lower motor neuron disease. Heredity 22 22588130
2012 A structural and functional analysis of Nna1 in Purkinje cell degeneration (pcd) mice. FASEB journal : official publication of the Federation of American Societies for Experimental Biology 21 22835831
2021 The Childhood-Onset Neurodegeneration with Cerebellar Atrophy (CONDCA) Disease Caused by AGTPBP1 Gene Mutations: The Purkinje Cell Degeneration Mouse as an Animal Model for the Study of this Human Disease. Biomedicines 18 34572343
2010 Abnormal sperm development in pcd(3J)-/- mice: the importance of Agtpbp1 in spermatogenesis. Molecules and cells 17 21110128
2020 Nna1 gene deficiency triggers Purkinje neuron death by tubulin hyperglutamylation and ER dysfunction. JCI insight 16 33004692
2010 A defect in cytosolic carboxypeptidase 1 (Nna1) causes autophagy in Purkinje cell degeneration mouse brain. Autophagy 14 20484972
2015 Mutagenesis Screen Identifies agtpbp1 and eps15L1 as Essential for T lymphocyte Development in Zebrafish. PloS one 13 26161877
2024 NIa-Pro of sugarcane mosaic virus targets Corn Cysteine Protease 1 (CCP1) to undermine salicylic acid-mediated defense in maize. PLoS pathogens 9 38484013
2023 Deleterious genetic changes in AGTPBP1 result in teratozoospermia with sperm head and flagella defects. Journal of cellular and molecular medicine 9 37937809
2012 The association of anti-CCP1 antibodies with disease activity score 28 (DAS-28) in rheumatoid arthritis. Advanced biomedical research 9 23210089
2022 Nna1, Essential for Purkinje Cell Survival, Is also Associated with Emotion and Memory. International journal of molecular sciences 8 36361749
2018 Ccp1 modulates epigenetic stability at centromeres and affects heterochromatin distribution in Schizosaccharomyces pombe. The Journal of biological chemistry 8 29899117
2010 Regulation of cell proliferation and apoptosis in neuroblastoma cells by ccp1, a FGF2 downstream gene. BMC cancer 8 21118521
2021 CCP1, a Tubulin Deglutamylase, Increases Survival of Rodent Spinal Cord Neurons following Glutamate-Induced Excitotoxicity. eNeuro 7 33688040
2015 Bioinformatics Data Mining Approach Suggests Coexpression of AGTPBP1 with an ALS-linked Gene C9orf72. Journal of central nervous system disease 7 26106267
2023 Circ-AGTPBP1 promotes white matter injury through miR-140-3p/Pcdh17 axis role of Circ-AGTPBP1 in white matter injury. Journal of bioenergetics and biomembranes 6 37994971
2021 The AGTPBP1 gene in neurobiology. Gene 6 34637898
2021 A novel pathogenic variant in the 3' end of the AGTPBP1 gene gives rise to neurodegeneration without cerebellar atrophy: an expansion of the disease phenotype? Neurogenetics 5 33909173
2021 Ccp1-Ndc80 switch at the N terminus of CENP-T regulates kinetochore assembly. Proceedings of the National Academy of Sciences of the United States of America 4 34810257
2015 Peroxynitrite and hydrogen peroxide elicit similar cellular stress responses mediated by the Ccp1 sensor protein. Free radical biology & medicine 4 25881547
2012 Analysis of the light-sensitivity of the photoreceptor cells of the ataxia and male sterility (AMS) mouse, an Nna1 mutant. Pathology international 4 23121602
2017 Construction of human MASP-2-CCP1/2SP, CCP2SP, SP plasmid DNA nanolipoplexes and the effects on tuberculosis in BCG-infected mice. Microbial pathogenesis 3 28578092
2023 CCP1, a Regulator of Tubulin Post-Translational Modifications, Potentially Plays an Essential Role in Cerebellar Development. International journal of molecular sciences 2 36982413
2025 Lacosamide Is a Novel Drug That Improves AGTPBP1 Knockout-Mediated Impairment of Neuronal and Dopaminergic Function. Molecular neurobiology 1 40347376
2025 CCP1 Inhibits Pulmonary Fibrosis by Suppressing Fibrotic Progression Through the EIF4B/PI3K/AKT Pathways. Cell biology international 1 40590568
2025 Single-Cell RNA Sequencing of Retina Reveals Nna1 Upregulation in Myopic Diabetic Retinopathy as a Protective Factor Against Diabetic Damage. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 1 41190783
2024 Set2 regulates Ccp1 and Swc2 to ensure centromeric stability by retargeting CENP-A. Nucleic acids research 1 38442274
2024 Targeting AGTPBP1 inhibits pancreatic cancer progression via regulating microtubules and ERK signaling pathway. Molecular medicine (Cambridge, Mass.) 1 39129004
2026 AGTPBP1 promotes breast cancer progression via the EVPL/ERK signaling axis. Experimental cell research 0 41905574
2026 A novel AGTPBP1-ERK/MYLK network mediates pancreatic cancer progression. Tissue & cell 0 41930881
2025 Ccp1 depletion disrupts network integration of hippocampal parvalbumin interneurons. iScience 0 40989022
2025 Human teratozoospermia-related AGTPBP1 R791H mutation is associated with sperm head and tail defects in a CRISPR-engineered murine model. Journal of assisted reproduction and genetics 0 41160201
2025 Model of selective neurodegeneration driven by a Ccp1 mutation leads to atypical microglia with an increased response to pathological stimuli. Brain, behavior, and immunity 0 41475674