Affinage

PLEC

Plectin · UniProt Q15149

Round 2 corrected
Length
4684 aa
Mass
531.8 kDa
Annotated
2026-04-28
130 papers in source corpus 15 papers cited in narrative 14 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

Plectin is a giant cytoskeletal linker protein that bridges intermediate filament networks (keratins, desmin, vimentin) to hemidesmosomes, the sarcolemma, and other membrane–cytoskeleton junctions, thereby maintaining mechanical integrity in skin, skeletal muscle, and heart (PMID:8894687, PMID:9701547). Loss-of-function mutations in common exons cause epidermolysis bullosa simplex with muscular dystrophy (EBS-MD), whereas isoform-specific mutations in exon 1f cause limb-girdle muscular dystrophy (LGMD2Q) and mutations in exon 1a cause skin-only EBS, demonstrating that distinct plectin isoforms fulfill tissue-restricted anchoring roles (PMID:21109228, PMID:25712130). Beyond structural scaffolding, plectin regulates actin stress fiber dynamics, anchors mitochondria to keratin 8 to facilitate mitophagy, and competitively displaces NRF2 from KEAP1 to activate antioxidant gene expression (PMID:9701547, PMID:33783309, PMID:39500864). Plectin also participates in NFκB1/CXCL9 signaling downstream of lipid mediator stimulation in breast cancer cells (PMID:39695149).

Mechanistic history

Synthesis pass · year-by-year structured walk · 8 steps
  1. 1996 High

    Identification of PLEC as the gene mutated in EBS-MD established that plectin is an essential cytoskeleton–membrane anchoring protein in both skin and muscle, resolving the molecular basis of combined epidermal fragility and muscular dystrophy.

    Evidence Mutation analysis, immunofluorescence, and electron microscopy in patient skin and muscle biopsies from multiple families

    PMID:8696340 PMID:8894687 PMID:8941634

    Open questions at the time
    • Mechanism by which plectin couples intermediate filaments to hemidesmosomal transmembrane proteins was not structurally resolved
    • Contribution of individual plectin isoforms to tissue-specific phenotypes was unknown
  2. 1998 High

    Plectin-null mice revealed that plectin binds all three major cytoskeletal networks and regulates actin stress fiber dynamics, expanding its role from a passive linker to an active regulator of cytoskeletal organization.

    Evidence Targeted gene inactivation in mice; phenotypic analysis and in vitro cell studies from plectin-null cells

    PMID:9701547

    Open questions at the time
    • Molecular mechanism of actin stress fiber regulation by plectin was not dissected
    • Relative contributions of intermediate filament anchoring versus actin regulation to the lethal phenotype were not separated
  3. 2010 High

    Isoform-specific mutations demonstrated that plectin isoform 1f (P1f) links the sarcolemma to the sarcomere, and its selective loss causes LGMD2Q without skin disease, proving that isoform diversity explains tissue-restricted pathology.

    Evidence Homozygosity mapping, PLEC sequencing, isoform-specific qRT-PCR, immunofluorescence, and TEM in patient muscle from multiple families

    PMID:21109228

    Open questions at the time
    • Structural basis for P1f-specific sarcolemma–sarcomere anchoring was not determined
    • Whether P1f loss also impairs signaling at the sarcolemma was not assessed
  4. 2015 High

    Disruption of plectin isoform 1a (P1a) alone caused skin-only EBS with hypoplastic hemidesmosomes, completing the isoform–tissue specificity map by showing P1a is the dominant epidermal basal cell isoform.

    Evidence DNA sequencing, immunofluorescence antigen mapping, TEM, and isoform expression profiling in patient skin and control tissues

    PMID:25712130

    Open questions at the time
    • Whether P1a-specific residues mediate unique protein–protein interactions at hemidesmosomes was not resolved
    • Functional compensation among isoforms in tissues co-expressing P1a and other variants was not tested
  5. 2021 Medium

    Discovery that plectin physically anchors mitochondria to keratin 8 and facilitates fission-mediated mitophagy revealed a non-structural role, explaining how plectin loss can cause mitochondrial dysfunction and necrotic cell death under oxidative stress.

    Evidence Reciprocal co-immunoprecipitation, siRNA knockdown of PLEC and KRT8, mitophagy flux assays, and live-cell imaging in retinal pigment epithelial cells

    PMID:33783309

    Open questions at the time
    • Domain on plectin responsible for mitochondrial anchoring was not mapped
    • Whether this mitochondrial-anchoring function is relevant in muscle or skin disease has not been tested
    • Confirmation in additional cell types is lacking
  6. 2023 Medium

    Plectin knockdown in the inner ear reduced synaptic mitochondrial potential and caused ribbon synapse loss, extending plectin's mitochondrial-support function to cochlear neurons and identifying biallelic PLEC variants as a cause of isolated hearing loss.

    Evidence Whole-exome sequencing in hearing-loss families, plectin knockdown in zebrafish and mouse inner ear, mitochondrial membrane potential assay, and ribbon synapse quantification

    PMID:37393735

    Open questions at the time
    • Specific plectin isoform(s) mediating cochlear function are unknown
    • Mechanism linking plectin to mitochondrial membrane potential at synapses was not dissected
  7. 2024 Medium

    Plectin was shown to competitively bind KEAP1 and displace NRF2, enabling NRF2 nuclear translocation and antioxidant gene activation, establishing plectin as a direct modulator of the oxidative stress response and radioresistance in ESCC.

    Evidence ChIP (ΔNp63α on PLEC promoter), co-IP (PLEC–KEAP1), subcellular fractionation, PLEC overexpression/knockdown with ROS measurement, and xenograft radiosensitivity assay

    PMID:39500864

    Open questions at the time
    • KEAP1-binding domain on plectin has not been mapped
    • Generalizability of the PLEC/KEAP1/NRF2 axis to non-cancer contexts is untested
    • Single-lab finding awaits independent replication
  8. 2024 Medium

    Placement of plectin upstream of NFκB1/CXCL9 in lipid-mediator-driven breast cancer signaling revealed yet another signaling axis influenced by plectin, beyond its structural and NRF2 roles.

    Evidence Transcriptomics/proteomics after EpOME treatment, PLEC siRNA knockdown, and in vivo xenograft/metastasis assays in TNBC models

    PMID:39695149

    Open questions at the time
    • Direct physical interaction between plectin and NFκB1 pathway components has not been demonstrated
    • Mechanism by which plectin activates NFκB1 is undefined
    • Single-lab study in one cancer type

Open questions

Synthesis pass · forward-looking unresolved questions
  • How plectin's structural scaffolding, mitochondrial anchoring, and newly discovered signaling roles (NRF2, NFκB1) are coordinated across tissues and whether they are functionally coupled or independent remain major open questions.
  • No high-resolution structure of full-length plectin or its isoform-specific domains exists
  • Domain-level separation of structural versus signaling functions has not been achieved
  • In vivo isoform-specific knockout models for signaling readouts are lacking

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0008092 cytoskeletal protein binding 6 GO:0005198 structural molecule activity 5 GO:0098772 molecular function regulator activity 2
Localization
GO:0005856 cytoskeleton 6 GO:0005886 plasma membrane 3 GO:0005739 mitochondrion 2 GO:0005829 cytosol 2
Pathway
R-HSA-1500931 Cell-Cell communication 3 R-HSA-8953897 Cellular responses to stimuli 3 R-HSA-1643685 Disease 2 R-HSA-397014 Muscle contraction 2 R-HSA-9612973 Autophagy 1
Partners
DESDSTITGB4KEAP1KRT8VIM
Complex memberships
Hemidesmosome

Evidence

Reading pass · 14 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1996 Plectin (encoded by PLEC1) is required for attachment of intermediate keratin filament networks to hemidesmosomal complexes in skin and for structural integrity of sarcolemma in muscle; homozygous deletion mutations in PLEC1 cause epidermolysis bullosa simplex with muscular dystrophy (EBS-MD), demonstrating plectin's critical role as a cytoskeleton-membrane anchorage protein in both epithelia and muscle. Immunofluorescence with anti-plectin antibody (HD-1), mutation analysis (sequencing of PLEC1 cDNA/genomic DNA), electron microscopy of patient skin Human molecular genetics High 8696340 8894687 8941634
1996 Absence of plectin in muscle fibers leads to aberrant localization of desmin, indicating plectin normally anchors desmin-containing intermediate filaments to the sarcolemma; a homozygous nonsense mutation in PLEC1 causes decay of aberrant plectin mRNA and loss of plectin protein. Immunofluorescence with plectin and desmin antibodies on patient muscle biopsies; RT-PCR and sequencing to detect nonsense-mediated mRNA decay The Journal of clinical investigation High 8941634
1998 Plectin and its isoforms function as versatile cytoskeletal linker proteins that bind to subcomponents of all three major cytoskeletal filament networks (intermediate filaments, actin, microtubules), the subplasma membrane protein skeleton, and plasma membrane-cytoskeleton junctional complexes; plectin-deficient mice generated by targeted gene inactivation die shortly after birth with severe defects in skin, skeletal muscle, and heart, and cells derived from these animals reveal an essential role for plectin as a regulator of actin stress fiber dynamics. Gene knockout (targeted inactivation), in vitro cell studies of plectin-null cells, biochemical binding studies Journal of cell science High 9701547
2003 Plectin was identified as a component that forms specific complexes with activated EGFR-Shc signaling complexes upon EGF stimulation; this was revealed by SILAC-based quantitative proteomics showing selective enrichment of plectin in Grb2-SH2 affinity purifications from EGF-stimulated cells, linking plectin to receptor tyrosine kinase signaling. SILAC quantitative proteomics, GST-SH2 affinity purification from EGF-stimulated vs. unstimulated HeLa cells Nature biotechnology Medium 12577067
2005 Plectin is a target of ISG15 conjugation in IFN-β-treated human cells, placing it within the ISG15-modified proteome that mediates innate immune responses. Double-affinity purification of ISG15-modified proteins from IFN-β-treated HeLa cells followed by mass spectrometry identification Proceedings of the National Academy of Sciences of the United States of America Low 16009940
2007 Spliceosome-mediated RNA trans-splicing (SMaRT) using a 5' pre-trans-splicing molecule encoding wild-type PLEC1 exons 2–9 specifically replaces the mutated 5' portion of the endogenous PLEC1 transcript in EBS-MD fibroblasts, reduces mutant mRNA levels, and restores wild-type plectin expression pattern, demonstrating that the dominant-negative leucine insertion in exon 9 causes plectin peptide chain aggregation and protein degradation. Transient transfection and retroviral transduction of EBS-MD fibroblasts with pre-trans-splicing molecules; immunofluorescence microscopy; Western blot quantification of plectin protein The Journal of investigative dermatology Medium 17989727
2010 A homozygous mutation in exon 1f of PLEC, which is isoform-specific to plectin isoform 1f (P1f), causes autosomal-recessive limb-girdle muscular dystrophy (LGMD2Q) without dermatological involvement; P1f deficiency breaks the linkage between the sarcolemma and sarcomere, resulting in ultrastructural alterations including membrane duplications, enlarged space between membrane and sarcomere, and Z-disk misalignment. SNP array homozygosity mapping, PLEC sequencing, RT-PCR/qRT-PCR for P1f mRNA expression, immunofluorescence of patient muscle, transmission electron microscopy American journal of human genetics High 21109228
2015 A nonsense mutation in exon 1a of PLEC, disrupting only plectin isoform 1a (P1a), causes autosomal-recessive skin-only EBS without extracutaneous involvement; P1a is dominantly expressed in epidermal basal cell layer and cultured keratinocytes, and its specific loss leads to hypoplastic hemidesmosomes and intra-epidermal cleavage while sparing heart and muscle that express other isoforms. DNA sequencing, immunofluorescence antigen mapping, transmission electron microscopy, Western blot, qRT-PCR on patient skin and cultured keratinocytes, control myocardium, and striated muscle Human molecular genetics High 25712130
2021 KRT8 (keratin 8) physically interacts with PLEC (plectin) to connect KRT8 to mitochondria; PLEC anchors mitochondria and mediates physical association with KRT8, facilitating mitochondrial fission-mediated mitophagy. Under oxidative stress, KRT8 phosphorylation diminishes this PLEC-anchoring association, impairing efficient mitophagy and leading to accumulation of damaged mitochondria and necrotic cell death in retinal pigment epithelial cells. Co-immunoprecipitation (KRT8-PLEC physical interaction), live-cell imaging, mitophagy flux assays, siRNA knockdown of KRT8 and PLEC, mitochondrial morphology/fission analysis, oxidative stress treatment Autophagy Medium 33783309
2024 ΔNp63α directly transactivates PLEC expression in esophageal squamous cell carcinoma (ESCC); PLEC competitively interacts with KEAP1, displacing NRF2 from the KEAP1 complex and enabling NRF2 nuclear translocation and activation of ROS-eliminating genes, thereby conferring radioresistance. Radiotherapy-induced ROS further activates ΔNp63α via NRF2, forming a positive feedback loop (ΔNp63α/PLEC/NRF2 axis). Chromatin immunoprecipitation (ΔNp63α binding to PLEC promoter), co-immunoprecipitation (PLEC-KEAP1 interaction), subcellular fractionation/immunofluorescence of NRF2 localization, PLEC overexpression/knockdown with ROS measurement, nude mouse xenograft radiosensitivity assay with NRF2 inhibitor Cell death & disease Medium 39500864
2023 Plectin knockdown in inner ear (zebrafish/mouse model) reduces synaptic mitochondrial potential and causes loss of ribbon synapses, indicating a role for plectin in maintaining neuronal/synaptic function in the cochlea; novel biallelic PLEC variants cause hearing loss in humans without skin or muscle involvement. Trio whole-exome sequencing, developmental expression analysis in mice and zebrafish, siRNA knockdown of plectin in inner ear with mitochondrial membrane potential assay and ribbon synapse quantification by immunofluorescence Hearing research Medium 37393735
2020 Plectin knockdown (via CRISPR/Cas9) in mesenchymal stem cells impacts Wnt signalling, glycosaminoglycan biosynthesis, and immune regulation pathways as revealed by RNA-sequencing, suggesting plectin modulates intracellular signaling responses to mechanical stimuli in joint tissues. CRISPR/Cas9 plectin knockdown in MSC line followed by RNA-sequencing pathway analysis Osteoarthritis and cartilage Low 32580029
2024 EpOMEs (epoxyoctadecenoic acids) upregulate PLEC expression in triple-negative breast cancer cells; upregulated PLEC in turn activates NFκB1, which transcriptionally regulates CXCL9, thereby promoting tumor growth and metastasis. PLEC knockdown inhibited EpOME-mediated promotion of TNBC, placing PLEC upstream of NFκB1/CXCL9 in this oncogenic signaling axis. Transcriptomics and proteomics after EpOME treatment, PLEC/CXCL9 siRNA knockdown, CYP2J2 overexpression/knockdown, in vivo xenograft and lung metastasis assays Cell death & disease Medium 39695149
2011 Plectin was identified as a component of the myosin-II-responsive focal adhesion proteome; its abundance in focal adhesions is enhanced by myosin II-mediated contractility, linking plectin to mechanosensing at focal adhesions. Proteomic analysis of isolated focal adhesions by mass spectrometry comparing myosin II-inhibited vs. control cells (blebbistatin treatment) Nature cell biology Low 21423176

Source papers

Stage 0 corpus · 130 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2010 Biological, clinical and population relevance of 95 loci for blood lipids. Nature 2873 20686565
2006 Global, in vivo, and site-specific phosphorylation dynamics in signaling networks. Cell 2861 17081983
2013 Discovery and refinement of loci associated with lipid levels. Nature genetics 2409 24097068
2012 Insights into RNA biology from an atlas of mammalian mRNA-binding proteins. Cell 1718 22658674
2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proceedings of the National Academy of Sciences of the United States of America 1479 12477932
2009 Defining the human deubiquitinating enzyme interaction landscape. Cell 1282 19615732
2004 Large-scale characterization of HeLa cell nuclear phosphoproteins. Proceedings of the National Academy of Sciences of the United States of America 1159 15302935
2015 The BioPlex Network: A Systematic Exploration of the Human Interactome. Cell 1118 26186194
2017 Architecture of the human interactome defines protein communities and disease networks. Nature 1085 28514442
2015 A human interactome in three quantitative dimensions organized by stoichiometries and abundances. Cell 1015 26496610
2005 Nucleolar proteome dynamics. Nature 934 15635413
2004 Immunoaffinity profiling of tyrosine phosphorylation in cancer cells. Nature biotechnology 916 15592455
2018 VIRMA mediates preferential m6A mRNA methylation in 3'UTR and near stop codon and associates with alternative polyadenylation. Cell discovery 829 29507755
2021 Dual proteome-scale networks reveal cell-specific remodeling of the human interactome. Cell 705 33961781
2012 A census of human soluble protein complexes. Cell 689 22939629
2011 Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Briefings in bioinformatics 656 21873635
2003 A proteomics strategy to elucidate functional protein-protein interactions applied to EGF signaling. Nature biotechnology 558 12577067
2011 Analysis of the myosin-II-responsive focal adhesion proteome reveals a role for β-Pix in negative regulation of focal adhesion maturation. Nature cell biology 490 21423176
2020 Mechanical regulation of glycolysis via cytoskeleton architecture. Nature 445 32051585
2005 Time-resolved mass spectrometry of tyrosine phosphorylation sites in the epidermal growth factor receptor signaling network reveals dynamic modules. Molecular & cellular proteomics : MCP 434 15951569
2022 OpenCell: Endogenous tagging for the cartography of human cellular organization. Science (New York, N.Y.) 432 35271311
2015 Panorama of ancient metazoan macromolecular complexes. Nature 407 26344197
1996 Normalization and subtraction: two approaches to facilitate gene discovery. Genome research 401 8889548
2005 Human ISG15 conjugation targets both IFN-induced and constitutively expressed proteins functioning in diverse cellular pathways. Proceedings of the National Academy of Sciences of the United States of America 383 16009940
2015 Proteome-wide profiling of protein assemblies by cross-linking mass spectrometry. Nature methods 370 26414014
1998 Role of plectin in cytoskeleton organization and dynamics. Journal of cell science 343 9701547
2021 A proximity-dependent biotinylation map of a human cell. Nature 339 34079125
2011 New gene functions in megakaryopoiesis and platelet formation. Nature 332 22139419
2010 Dynamics of cullin-RING ubiquitin ligase network revealed by systematic quantitative proteomics. Cell 318 21145461
1996 Plectin deficiency results in muscular dystrophy with epidermolysis bullosa. Nature genetics 314 8696340
1996 Homozygous deletion mutations in the plectin gene (PLEC1) in patients with epidermolysis bullosa simplex associated with late-onset muscular dystrophy. Human molecular genetics 134 8894687
2001 Formation of PCDF, PCDD, PCB, and PCN in de novo synthesis from PAH: mechanistic aspects and correlation to fluidized bed incinerators. Chemosphere 126 11513122
2019 Development of a protein-ligand extended connectivity (PLEC) fingerprint and its application for binding affinity predictions. Bioinformatics (Oxford, England) 111 30202917
2003 The Caulobacter crescentus polar organelle development protein PodJ is differentially localized and is required for polar targeting of the PleC development regulator. Molecular microbiology 94 12581350
1996 A homozygous nonsense mutation in the PLEC1 gene in patients with epidermolysis bullosa simplex with muscular dystrophy. The Journal of clinical investigation 91 8941634
2010 Mutation in exon 1f of PLEC, leading to disruption of plectin isoform 1f, causes autosomal-recessive limb-girdle muscular dystrophy. American journal of human genetics 83 21109228
2014 Reducing NO and N₂O emission during aerobic denitrification by newly isolated Pseudomonas stutzeri PCN-1. Bioresource technology 79 24747385
1989 Turning off flagellum rotation requires the pleiotropic gene pleD: pleA, pleC, and pleD define two morphogenic pathways in Caulobacter crescentus. Journal of bacteriology 77 2536661
2023 Self-Driven Electron Transfer Biomimetic Enzymatic Catalysis of Bismuth-Doped PCN-222 MOF for Rapid Therapy of Bacteria-Infected Wounds. ACS nano 76 36622022
2017 A Missense Variant in PLEC Increases Risk of Atrial Fibrillation. Journal of the American College of Cardiology 68 29050564
2017 Effect of sulfamethoxazole on aerobic denitrification by strain Pseudomonas stutzeri PCN-1. Bioresource technology 63 28376383
2021 Understanding disorder and linker deficiency in porphyrinic zirconium-based metal-organic frameworks by resolving the Zr8O6 cluster conundrum in PCN-221. Nature communications 58 34035286
2015 Interaction of Cr(VI) reduction and denitrification by strain Pseudomonas aeruginosa PCN-2 under aerobic conditions. Bioresource technology 57 25795449
2007 5' trans-splicing repair of the PLEC1 gene. The Journal of investigative dermatology 57 17989727
2005 Epidermolysis bullosa simplex associated with pyloric atresia is a novel clinical subtype caused by mutations in the plectin gene (PLEC1). The Journal of molecular diagnostics : JMD 56 15681471
1989 Human fetal and adult liver metabolism of ethylmorphine. Relation to immunodetected cytochrome P-450 PCN and interactions with important fetal corticosteroids. Biochemical pharmacology 54 2818616
2013 The DivJ, CbrA and PleC system controls DivK phosphorylation and symbiosis in Sinorhizobium meliloti. Molecular microbiology 52 23909720
2008 The Anaplasma phagocytophilum PleC histidine kinase and PleD diguanylate cyclase two-component system and role of cyclic Di-GMP in host cell infection. Journal of bacteriology 47 18978058
2023 A novel CRISPR/Cas14a-based electrochemical biosensor for ultrasensitive detection of Burkholderia pseudomallei with PtPd@PCN-224 nanoenzymes for signal amplification. Biosensors & bioelectronics 46 36701949
2019 Prioritization of PLEC and GRINA as Osteoarthritis Risk Genes Through the Identification and Characterization of Novel Methylation Quantitative Trait Loci. Arthritis & rheumatology (Hoboken, N.J.) 43 30730609
2015 Mutation in exon 1a of PLEC, leading to disruption of plectin isoform 1a, causes autosomal-recessive skin-only epidermolysis bullosa simplex. Human molecular genetics 41 25712130
2016 Effects of heavy metals on aerobic denitrification by strain Pseudomonas stutzeri PCN-1. Applied microbiology and biotechnology 39 27853857
2013 Epidermolysis bullosa simplex with PLEC mutations: new phenotypes and new mutations. The British journal of dermatology 36 23289980
2020 Multi-tissue epigenetic analysis of the osteoarthritis susceptibility locus mapping to the plectin gene PLEC. Osteoarthritis and cartilage 35 32580029
2010 Congenital myasthenic syndrome associated with epidermolysis bullosa caused by homozygous mutations in PLEC1 and CHRNE. Clinical genetics 34 21175599
2021 KRT8 (keratin 8) attenuates necrotic cell death by facilitating mitochondrial fission-mediated mitophagy through interaction with PLEC (plectin). Autophagy 33 33783309
2010 Congenital muscular dystrophy, myasthenic symptoms and epidermolysis bullosa simplex (EBS) associated with mutations in the PLEC1 gene encoding plectin. Neuromuscular disorders : NMD 33 20624679
1989 Evidence for a PCN-P450 enzyme in chickens and comparison of its development with that of other phenobarbital-inducible forms. Molecular pharmacology 30 2725470
2016 Potential application of aerobic denitrifying bacterium Pseudomonas aeruginosa PCN-2 in nitrogen oxides (NOx) removal from flue gas. Journal of hazardous materials 29 27469045
2017 Effects of ZnO nanoparticles on aerobic denitrification by strain Pseudomonas stutzeri PCN-1. Bioresource technology 28 28500885
2012 Repeated dose toxicity and relative potency of 1,2,3,4,6,7-hexachloronaphthalene (PCN 66) 1,2,3,5,6,7-hexachloronaphthalene (PCN 67) compared to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) for induction of CYP1A1, CYP1A2 and thymic atrophy in female Harlan Sprague-Dawley rats. Toxicology 28 22813907
2002 Expression of rat Multidrug Resistance Protein 2 (Mrp2) in male and female rats during normal and pregnenolone-16alpha-carbonitrile (PCN)-induced postnatal ontogeny. Toxicology 27 12167307
2022 A New Paradigm in Pincer Iridium Chemistry: PCN Complexes for (De)Hydrogenation Catalysis and Beyond. Accounts of chemical research 26 35852837
2004 Promotion of thyroid tumors in rats by pregnenolone-16alpha-carbonitrile (PCN) and polychlorinated biphenyl (PCB). Toxicological sciences : an official journal of the Society of Toxicology 25 15201439
1997 Roles of the histidine protein kinase pleC in Caulobacter crescentus motility and chemotaxis. Journal of bacteriology 25 9294444
1985 Age- and sex-dependent induction of liver microsomal benzo[a]pyrene hydroxylase activity in rats treated with pregnenolone-16 alpha-carbonitrile (PCN). Carcinogenesis 25 3986963
2018 Whole exome sequencing identifies PLEC, EXO5 and DNAH7 as novel susceptibility genes in testicular cancer. International journal of cancer 24 29761480
2015 Phenazine-1-carboxamide (PCN) from Pseudomonas sp. strain PUP6 selectively induced apoptosis in lung (A549) and breast (MDA MB-231) cancer cells by inhibition of antiapoptotic Bcl-2 family proteins. Apoptosis : an international journal on programmed cell death 23 25820140
2014 Left ventricular non-compaction cardiomyopathy associated with epidermolysis bullosa simplex with muscular dystrophy and PLEC1 mutation. Neuromuscular disorders : NMD 23 25454730
1988 Monoclonal antibody directed detection of cytochrome P-450 (PCN) in human fetal liver. Biochemical pharmacology 20 3264502
2021 Insights into the Interaction between Immobilized Biocatalysts and Metal-Organic Frameworks: A Case Study of PCN-333. JACS Au 19 34977888
2024 Highly sensitive and selective demethylase FTO detection using a DNAzyme-mediated CRISPR/Cas12a signal cascade amplification electrochemiluminescence biosensor with C-CN/PCNV heterojunction as emitter. Biosensors & bioelectronics 18 38599073
2022 Regulation of the activity of the bacterial histidine kinase PleC by the scaffolding protein PodJ. The Journal of biological chemistry 18 35124010
2021 An S-ribonuclease binding protein EBS1 and brassinolide signaling are specifically required for Arabidopsis tolerance to bicarbonate. Journal of experimental botany 18 33165537
2020 Four Individuals with a Homozygous Mutation in Exon 1f of the PLEC Gene and Associated Myasthenic Features. Genes 18 32605089
2017 Glu20Ter Variant in PLEC 1f Isoform Causes Limb-Girdle Muscle Dystrophy with Lung Injury. Frontiers in neurology 18 28824526
2022 Mutation update: The spectra of PLEC sequence variants and related plectinopathies. Human mutation 17 35815343
2016 Epidermolysis bullosa simplex in sibling Eurasier dogs is caused by a PLEC non-sense variant. Veterinary dermatology 15 27878870
2018 A novel PLEC nonsense homozygous mutation (c.7159G > T; p.Glu2387*) causes epidermolysis bullosa simplex with muscular dystrophy and diffuse alopecia: a case report. BMC dermatology 14 29352809
2021 Circular RNA PLEC acts as a sponge of microRNA-198 to promote gastric carcinoma cell resistance to paclitaxel and tumorigenesis. Pathology, research and practice 13 34225215
2015 Germline copy number loss of UGT2B28 and gain of PLEC contribute to increased human esophageal squamous cell carcinoma risk in Southwest China. American journal of cancer research 13 26693059
2024 Sustainable Gold Nanoparticle (Au-NP) Growth within Interspaces of Porphyrinic Zirconium-Based Metal-Organic Frameworks: Green Synthesis of PCN-224/Au-NPs and Its Anticancer Effect on Colorectal Cancer Cells Assay. ACS applied materials & interfaces 12 38194287
2018 Matrimid-JUC-62 and Matrimid-PCN-250 mixed matrix membranes displaying light-responsive gas separation and beneficial ageing characteristics for CO2/N2 separation. Scientific reports 12 29440732
2024 A Zn-modified PCN-224 fluorescent nanoprobe for selective and sensitive turn-on detection of glutathione. Talanta 11 38199125
2024 Strong cathode electroluminescence biosensor based on CeO2 functionalized PCN-222@Ag NPs for sensitive detection of p-Tau-181 protein. Journal of colloid and interface science 11 38520931
2023 Reprogramming of the tumor microenvironment using a PCN-224@IrNCs/D-Arg nanoplatform for the synergistic PDT, NO, and radiosensitization therapy of breast cancer and improving anti-tumor immunity. Nanoscale 11 37318099
2017 Novel compound heterozygous PLEC mutations lead to early‑onset limb‑girdle muscular dystrophy 2Q. Molecular medicine reports 11 28447722
2016 Differences in the action of lower and higher chlorinated polychlorinated naphthalene (PCN) congeners on estrogen dependent breast cancer cell line viability and apoptosis, and its correlation with Ahr and CYP1A1 expression. Toxicology 11 27501765
2025 Novel Pt@PCN-Cu-induced cuproptosis amplifies αPD-L1 immunotherapy in pancreatic ductal adenocarcinoma through mitochondrial HK2-mediated PD-L1 upregulation. Journal of experimental & clinical cancer research : CR 10 40382627
2023 Pharmacological profiles and anti-inflammatory activity of pCN-diEPP and mCN-diEPP, new alpha9alpha10 nicotinic receptor ligands. Neuropharmacology 10 37758018
2022 Evaluation of Systemic Gentamicin as Translational Readthrough Therapy for a Patient With Epidermolysis Bullosa Simplex With Muscular Dystrophy Owing to PLEC1 Pathogenic Nonsense Variants. JAMA dermatology 10 35234827
2022 PCN-Miner: an open-source extensible tool for the analysis of Protein Contact Networks. Bioinformatics (Oxford, England) 10 35799364
2021 Disposable biosensor based on novel ternary Ru-PEI@PCN-333(Al) self-enhanced electrochemiluminescence system for on-site determination of caspase-3 activity. Talanta 10 34861485
2021 PCN-224 Nanoparticle/Polyacrylonitrile Nanofiber Membrane for Light-Driven Bacterial Inactivation. Nanomaterials (Basel, Switzerland) 10 34947511
2016 Identification of Candidate Target Cyp Genes for microRNAs Whose Expression Is Altered by PCN and TCPOBOP, Representative Ligands of PXR and CAR. Biological & pharmaceutical bulletin 10 27237601
2015 Reduction of PCN biosynthesis by NO in Pseudomonas aeruginosa. Redox biology 10 26874276
2014 Compound heterozygous PLEC mutations in a patient of consanguineous parentage with epidermolysis bullosa simplex with muscular dystrophy and diffuse alopecia. International journal of dermatology 10 25209331
2023 Effects of Copy Number Variations in the Plectin (PLEC) Gene on the Growth Traits and Meat Quality of Leizhou Black Goats. Animals : an open access journal from MDPI 9 38067002
2022 Highly water-dispersible PCN nanosheets as light-controlled lysosome self-promoting escape type non-cationic gene carriers for tumor therapy. Journal of materials chemistry. B 9 35775960
2020 Cellular evaluation of the metal-organic framework PCN-224 associated with inflammation and autophagy. Toxicology in vitro : an international journal published in association with BIBRA 9 33058999
2018 Cell cycle accumulation of the proliferating cell nuclear antigen PCN-1 transitions from continuous in the adult germline to intermittent in the early embryo of C. elegans. BMC developmental biology 9 29848313
2024 ΔNp63α promotes radioresistance in esophageal squamous cell carcinoma through the PLEC-KEAP1-NRF2 feedback loop. Cell death & disease 8 39500864
2022 Insight into sulfamethoxazole effects on aerobic denitrification by strain Pseudomonas aeruginosa PCN-2: From simultaneous degradation performance to transcriptome analysis. Chemosphere 8 36493888
2015 Dynamical Localization of DivL and PleC in the Asymmetric Division Cycle of Caulobacter crescentus: A Theoretical Investigation of Alternative Models. PLoS computational biology 8 26186202
2024 Epoxy metabolites of linoleic acid promote the development of breast cancer via orchestrating PLEC/NFκB1/CXCL9-mediated tumor growth and metastasis. Cell death & disease 7 39695149
2013 Role of helical constraints of the EBS1-IBS1 duplex of a group II intron on demarcation of the 5' splice site. RNA (New York, N.Y.) 7 24243113
2024 Metal-Organic Framework PCN-224 Combined Cobalt Oxide Nanoparticles for Hypoxia Relief and Synergistic Photodynamic/Chemodynamic Therapy. Chemistry (Weinheim an der Bergstrasse, Germany) 6 38606488
2019 Epigenetic Memory Is Involved in the Persistent Alterations of Drug-Processing Genes in Adult Mice Due to PCN-Activated PXR During Early Life. Toxicological sciences : an official journal of the Society of Toxicology 5 31388680
2016 Epidermolysis bullosa simplex with muscular dystrophy associated with PLEC deletion mutation. Neurology. Genetics 5 27766310
2025 A split-type photoelectrochemical sensor based on In2S3/PCN-224 Z-scheme heterojunction for ultrasensitive detection of ampicillin. Mikrochimica acta 4 39934406
2023 Delivery of Immobilized IFN-γ With PCN-333 and Its Effect on Human Mesenchymal Stem Cells. ACS biomaterials science & engineering 4 36598843
2023 Exploring the role of sandwich-type polyoxometalates in {K10(PW9O34)2M4(H2O)2}@PCN-222 (M = Mn, Ni, Zn) for electroreduction of CO2 to CO. Dalton transactions (Cambridge, England : 2003) 4 37470104
2023 "Gold-plated" PCN-222(Fe) and superconductive carbon black-based sandwich-type immunosensor for detecting CYFRA21-1. Journal of materials chemistry. B 4 37578169
2022 Epidermolysis Bullosa With Congenital Absence of Skin: Congenital Corneal Cloudiness and Esophagogastric Obstruction Including Extended Genotypic Spectrum of PLEC, LAMC2, ITGB4 and COL7A1. Frontiers in genetics 4 35432467
2022 Long-term treatment with the mPXR agonist PCN promotes hepatomegaly and lipid accumulation without hepatocyte proliferation in mice. Acta pharmacologica Sinica 4 35773338
2021 Congenital myopathy and epidermolysis bullosa due to PLEC variant. Neuromuscular disorders : NMD 4 34702657
2025 Self-driven charge transfer mechanism of Bi NPs/PCN-224 for enhanced photodynamic antimicrobial chemotherapy effect. Journal of colloid and interface science 3 40054254
2012 Functional analysis of the single Est1/Ebs1 homologue in Kluyveromyces lactis reveals roles in both telomere maintenance and rapamycin resistance. Eukaryotic cell 3 22544908
1976 Effect of pregnenolone-16alpha-carbonitrile (PCN) on rat liver. Acta hepato-gastroenterologica 3 970082
2025 Prioritising relevant polychlorinated naphthalene (PCN) congeners for human dietary exposure studies. Chemosphere 2 39733955
2023 Novel biallelic variants in the PLEC gene are associated with severe hearing loss. Hearing research 2 37393735
2023 Nitrogen-doped Zn/Fe@PCN derived from metal-organic frameworks activating persulfate to efficiently degrade rhodamine B. Environmental science and pollution research international 2 38055169
2022 Epidermolysis Bullosa: A Report of Three Cases with Novel Heterozygous Deletions in PLEC and Homozygous Non sense Mutations in COL7A1 Genes. Indian journal of dermatology 2 35656234
2022 Novel compound heterozygous mutations in the PLEC gene in a neonate with epidermolysis bullosa simplex with pyloric atresia. The Journal of dermatology 2 35996939
2019 Rational modifications of PCN-700 to induce electrical conductivity: a computational study. Dalton transactions (Cambridge, England : 2003) 2 31793579
2009 Exclusion of EGFR, HRAS, DSP, JUP, CTNNB1, PLEC1, and EPPK1 as functional candidate genes in 7 families with syndromic diarrhoea. Journal of pediatric gastroenterology and nutrition 2 19322062
2025 A review on multi-enzyme activity of ZIF, PBA, PCN and MIL MOF-derived cascade nano-enzymes for catalytic applications. Nanoscale 1 41143710
2023 Identification of genes governing resistance to PCN (Globodera rostochiensis) through transcriptome analysis in Solanum tuberosum. Functional & integrative genomics 1 37453957
2007 [Construction and verification of the dual-promoter plasmid pCN-SSISG as bivalent anti-caries DNA vaccine]. Shanghai kou qiang yi xue = Shanghai journal of stomatology 1 17546391
1987 Effect of age on the induction of in vitro drug metabolism by pregnenolone-16 alpha-carbonitrile (PCN); effect of age and PCN on immune responses. Die Pharmazie 1 3325990