Affinage

FBN2

Fibrillin-2 · UniProt P35556

Length
2912 aa
Mass
314.8 kDa
Annotated
2026-04-28
100 papers in source corpus 9 papers cited in narrative 9 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

Fibrillin-2 is a large extracellular matrix glycoprotein that polymerizes into microfibrils and functions as a critical scaffold for sequestering BMP and TGF-β ligands in a latent state, thereby regulating growth factor signaling during embryonic and postnatal development. In Fbn2-null mice, loss of fibrillin-2 leads to abnormally activated BMP signaling in skeletal muscle—a phenotype rescued by Bmp7 haploinsufficiency—and causes anterior segment dysgenesis in the eye including iris coloboma and disorganized zonular fibers (PMID:26114882, PMID:24130178). Missense mutations disrupting cysteine residues within EGF-like repeats encoded by exons 23–34 of FBN2 cause congenital contractural arachnodactyly, and FBN2 haploinsufficiency contributes to digit contractures and auricular anomalies in 5q23 deletion syndromes (PMID:7493032, PMID:11754102, PMID:25195018). Epigenetic silencing of FBN2 by promoter DNA methylation occurs in non-small cell lung cancer, where demethylation restores expression (PMID:15951052).

Mechanistic history

Synthesis pass · year-by-year structured walk · 6 steps
  1. 1995 High

    Identifying FBN2 as the disease gene for congenital contractural arachnodactyly established that fibrillin-2 is a functional component of extracellular microfibrils whose EGF-like domains are essential for musculoskeletal development.

    Evidence Sequencing of FBN2 in CCA patients identified cysteine-disrupting missense mutations in EGF-like repeats

    PMID:7493032

    Open questions at the time
    • No biochemical demonstration of how cysteine substitutions disrupt microfibril assembly
    • Signaling consequences of FBN2 mutations were unknown
    • Functional redundancy with FBN1 not addressed
  2. 2002 Medium

    Systematic mutation mapping revealed that CCA mutations cluster in exons 23–34 and, unlike FBN1 mutations, do not target calcium-binding consensus sequences, indicating distinct pathogenic mechanisms for the two fibrillins.

    Evidence SSCP analysis and direct sequencing of exons 22–36 in CCA patients across two studies identified a concentrated mutation hotspot

    PMID:11754102 PMID:9714438

    Open questions at the time
    • No structural or biophysical explanation for why non-calcium-binding residues are preferentially affected
    • Functional significance of the exon 23–34 region beyond genotype–phenotype correlation not tested
  3. 2005 Medium

    Demonstrating promoter methylation-mediated silencing of FBN2 in lung cancer revealed that loss of this ECM component is an epigenetically regulated event in tumorigenesis, extending FBN2's relevance beyond connective tissue disorders.

    Evidence Methylation-specific PCR and 5-aza-2'-deoxycytidine rescue of FBN2 expression in NSCLC cell lines and primary tumors

    PMID:15951052

    Open questions at the time
    • Whether restored FBN2 expression suppresses tumor growth was not tested
    • Mechanism by which FBN2 silencing promotes tumorigenesis unknown
  4. 2013 High

    Analysis of Fbn2-null mice demonstrated that fibrillin-2 is the dominant fibrillin during eye development and is indispensable for optic fissure closure and zonular fiber organization, with only partial compensation by upregulated fibrillin-1.

    Evidence Confocal immunofluorescence and histology of Fbn2−/− mouse eyes showing iris coloboma, ciliary body hypoplasia, and disorganized microfibrils

    PMID:24130178

    Open questions at the time
    • Whether BMP or TGF-β signaling is dysregulated in the Fbn2-null eye was not examined
    • Relevance to human ocular disease beyond CCA not established at this point
  5. 2014 Medium

    Multiple genetic studies extended fibrillin-2's developmental roles: FBN2 haploinsufficiency in 5q23 deletions explained digit and ear anomalies, rare FBN2 variants localized to Bruch's membrane were linked to macular degeneration, and rare FBN2 variants in scoliosis patients were associated with TGF-β pathway upregulation.

    Evidence Array CGH in deletion patients with expression analysis (digit/ear phenotype); exome sequencing with immunolocalization in human eye sections (maculopathy); exome sequencing with TGF-β pathway analysis in AIS cohorts

    PMID:24833718 PMID:24899048 PMID:25195018

    Open questions at the time
    • Functional validation of specific FBN2 variants (e.g., p.Glu1144Lys) has not been performed
    • Direct measurement of TGF-β or BMP sequestration by fibrillin-2 in these tissue contexts is lacking
    • Mechanisms connecting FBN2 loss to scoliosis pathogenesis remain correlative
  6. 2015 High

    Genetic epistasis in Fbn2-null mice proved that fibrillin-2 sequesters BMP7 in a latent ECM complex to regulate postnatal muscle development, directly linking microfibril function to growth factor bioavailability.

    Evidence Fbn2-null mice showed reduced muscle mass with activated BMP signaling; Bmp7 heterozygous deletion rescued muscle mass and fat phenotypes; in vitro BMP signaling assays

    PMID:26114882

    Open questions at the time
    • Direct biochemical interaction between fibrillin-2 and BMP7 propeptide complexes not reconstituted
    • Whether fibrillin-2 also sequesters other BMP family members in muscle ECM is unknown
    • Structural basis for fibrillin-2 preferentially sequestering BMPs versus TGF-β ligands not resolved

Open questions

Synthesis pass · forward-looking unresolved questions
  • The structural basis for fibrillin-2's selective sequestration of BMP versus TGF-β ligands, the molecular mechanism by which exon 23–34 mutations dominantly disrupt microfibril function, and whether FBN2 silencing functionally contributes to cancer progression remain unresolved.
  • No reconstituted fibrillin-2–BMP complex structure exists
  • Dominant-negative versus haploinsufficiency mechanisms for CCA mutations not distinguished
  • Tumor-suppressive role of FBN2 not functionally validated

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0005198 structural molecule activity 2 GO:0098772 molecular function regulator activity 2
Localization
GO:0031012 extracellular matrix 4 GO:0005576 extracellular region 2
Pathway
R-HSA-1474244 Extracellular matrix organization 3 R-HSA-1266738 Developmental Biology 2 R-HSA-162582 Signal Transduction 2
Partners
BMP7FBN1
Complex memberships
microfibrils

Evidence

Reading pass · 9 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1995 Missense mutations in FBN2 that substitute cysteine residues in EGF-like (epidermal growth-factor-like) repeats cause congenital contractural arachnodactyly (CCA), establishing that fibrillin-2 is a structural component of extracellular matrix microfibrils and that its EGF-like domains are critical for function. Identification of FBN2 missense mutations in CCA patients by sequencing; functional inference from domain architecture Nature genetics High 7493032
1998 CCA-causing FBN2 mutations cluster specifically in the region encoded by exons 24–34, mirroring the neonatal severe-MFS mutation cluster in FBN1, indicating this central fibrillin-2 domain plays a critical role in human embryogenesis and musculoskeletal development. RT-PCR mutation screening of FBN2 mRNA from CCA patient cell lines; genotype-phenotype correlation American journal of medical genetics Medium 9714438
2002 Ten novel FBN2 mutations causing CCA cluster between exons 23 and 34, and notably none alter the calcium-binding consensus sequence in EGF-like domains (in contrast to FBN1 mutations), indicating distinct mechanisms of fibrillin-1 vs fibrillin-2 pathogenesis and revealing that the calcium-binding EGF module is not the primary functional target in fibrillin-2. SSCP analysis and direct sequencing of exons 22–36 of FBN2 in CCA patients; genotype-phenotype analysis Human mutation Medium 11754102
2013 Fibrillin-2 is the dominant fibrillin isoform expressed during mouse eye development; Fbn2-null mice display anterior segment dysgenesis including iris coloboma, ciliary body hypoplasia, and disorganized zonular fibers, demonstrating an indispensable role for fibrillin-2 in ocular morphogenesis and optic fissure closure. Loss of Fbn2 is partially compensated by increased fibrillin-1 expression, but resulting microfibrils are disorganized. Confocal microscopy with antibodies against microfibril components in Fbn2−/− mice; histological analysis Investigative ophthalmology & visual science High 24130178
2015 Loss of fibrillin-2 in Fbn2 null mice causes reduced muscle mass, abnormal muscle histology, and abnormally activated BMP signaling in skeletal muscle; genetic rescue by deletion of a single Bmp7 allele rescues reduced muscle mass and white fat accumulation, demonstrating that fibrillin-2 sequesters BMP complexes in a latent state in the extracellular matrix to regulate BMP signaling during postnatal muscle development. Fbn2 null mouse phenotyping (creatine kinase assays, histology, myosin isoform analysis); genetic epistasis by Bmp7 heterozygous deletion; in vitro BMP signaling assays PLoS genetics High 26114882
2014 FBN2 localizes to Bruch's membrane in the human eye, and its expression is reduced in aging and AMD eyes; rare variants in FBN2 (including p.Glu1144Lys) segregate with autosomal dominant maculopathy, implicating fibrillin-2 in maintenance of blood-retina homeostasis through the elastin microfibril ECM of Bruch's membrane. Whole exome sequencing and Sanger validation in family; immunolocalization of FBN2 in human eye sections; genetic association in 10,337 AMD cases and 11,174 controls Human molecular genetics Medium 24899048
2014 Rare variants in FBN2 are enriched in severely affected adolescent idiopathic scoliosis (AIS) cases and are associated with upregulation of the TGF-β pathway, extending the biological role of fibrillin-2 to spinal curvature regulation through TGF-β signaling control. Exome sequencing and sequencing of FBN2 in 852 AIS cases and 669 controls; TGF-β pathway activation analysis; replication in Han Chinese cohort Human molecular genetics Medium 24833718
2005 FBN2 promoter methylation leads to silencing of FBN2 expression in non-small cell lung cancer cells; treatment with the demethylating agent 5-aza-2'-deoxycytidine restores FBN2 expression, establishing that FBN2 is epigenetically silenced by DNA methylation in NSCLC. RT-PCR, methylation-specific PCR, and 5-aza-2'-deoxycytidine demethylation treatment of NSCLC cell lines and primary tumors Lung cancer (Amsterdam, Netherlands) Medium 15951052
2014 Haploinsufficiency for FBN2 in 5q23 deletions accounts for the digital (finger contractures) and auricular features (crumpled ear helices) in a syndromic form of Pierre Robin sequence, establishing a developmental role for fibrillin-2 in digit and ear morphogenesis. Clinical array-based comparative genome hybridisation (aCGH) in patients with 5q22–5q31 deletions; expression analysis of orthologous genes in embryonic mice European journal of medical genetics Medium 25195018

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2018 cHCC-CCA: Consensus terminology for primary liver carcinomas with both hepatocytic and cholangiocytic differentation. Hepatology (Baltimore, Md.) 268 29360137
1995 Fibrillin-2 (FBN2) mutations result in the Marfan-like disorder, congenital contractural arachnodactyly. Nature genetics 221 7493032
1991 Sites of interaction of the CCA end of peptidyl-tRNA with 23S rRNA. Proceedings of the National Academy of Sciences of the United States of America 134 2023922
2003 Endonucleolytic processing of CCA-less tRNA precursors by RNase Z in Bacillus subtilis. The EMBO journal 115 12941704
2004 Mechanism of transfer RNA maturation by CCA-adding enzyme without using an oligonucleotide template. Nature 108 15295590
2002 Ten novel FBN2 mutations in congenital contractural arachnodactyly: delineation of the molecular pathogenesis and clinical phenotype. Human mutation 101 11754102
2014 Rare variants in FBN1 and FBN2 are associated with severe adolescent idiopathic scoliosis. Human molecular genetics 98 24833718
1984 Cleavage of tRNA precursors by the RNA subunit of E. coli ribonuclease P (M1 RNA) is influenced by 3'-proximal CCA in the substrates. Cell 85 6380759
2009 UMD-predictor, a new prediction tool for nucleotide substitution pathogenicity -- application to four genes: FBN1, FBN2, TGFBR1, and TGFBR2. Human mutation 77 19370756
2005 A story with a good ending: tRNA 3'-end maturation by CCA-adding enzymes. Current opinion in structural biology 70 16364630
1998 The CCA-adding enzyme has a single active site. The Journal of biological chemistry 70 9792681
2003 Crystal structure of the human CCA-adding enzyme: insights into template-independent polymerization. Journal of molecular biology 68 12729736
2001 Collaboration between CC- and A-adding enzymes to build and repair the 3'-terminal CCA of tRNA in Aquifex aeolicus. Science (New York, N.Y.) 66 11701927
2010 CCA addition to tRNA: implications for tRNA quality control. IUBMB life 65 20101632
2021 WDR5 facilitates EMT and metastasis of CCA by increasing HIF-1α accumulation in Myc-dependent and independent pathways. Molecular therapy : the journal of the American Society of Gene Therapy 64 33601056
2015 The 3' addition of CCA to mitochondrial tRNASer(AGY) is specifically impaired in patients with mutations in the tRNA nucleotidyl transferase TRNT1. Human molecular genetics 63 25652405
2006 Complete crystallographic analysis of the dynamics of CCA sequence addition. Nature 63 17051158
2003 Crystal structures of an archaeal class I CCA-adding enzyme and its nucleotide complexes. Molecular cell 61 14636575
1998 A top-half tDNA minihelix is a good substrate for the eubacterial CCA-adding enzyme. RNA (New York, N.Y.) 61 9510330
2005 The C. elegans T-type calcium channel CCA-1 boosts neuromuscular transmission. The Journal of experimental biology 57 15914662
2004 A pathogenesis-associated mutation in human mitochondrial tRNALeu(UUR) leads to reduced 3'-end processing and CCA addition. Journal of molecular biology 55 15019775
2017 miRNA and lncRNA as biomarkers in cholangiocarcinoma(CCA). Oncotarget 52 29246025
2014 Rare and common variants in extracellular matrix gene Fibrillin 2 (FBN2) are associated with macular degeneration. Human molecular genetics 52 24899048
1998 Clustering of FBN2 mutations in patients with congenital contractural arachnodactyly indicates an important role of the domains encoded by exons 24 through 34 during human development. American journal of medical genetics 52 9714438
2009 The FBN2 gene: new mutations, locus-specific database (Universal Mutation Database FBN2), and genotype-phenotype correlations. Human mutation 49 18767143
2009 Methylation of CLDN6, FBN2, RBP1, RBP4, TFPI2, and TMEFF2 in esophageal squamous cell carcinoma. Oncology reports 49 19288010
2015 Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice. PLoS genetics 48 26114882
2009 Mechanism for the definition of elongation and termination by the class II CCA-adding enzyme. The EMBO journal 48 19745807
2004 Children's exposure to arsenic from CCA-treated wooden decks and playground structures. Risk analysis : an official publication of the Society for Risk Analysis 48 15028000
2003 Divergent evolutions of trinucleotide polymerization revealed by an archaeal CCA-adding enzyme structure. The EMBO journal 48 14592988
2006 The precursor tRNA 3'-CCA interaction with Escherichia coli RNase P RNA is essential for catalysis by RNase P in vivo. RNA (New York, N.Y.) 43 17135488
2005 Aberrant methylation of FBN2 in human non-small cell lung cancer. Lung cancer (Amsterdam, Netherlands) 43 15951052
2004 Exchange of regions between bacterial poly(A) polymerase and the CCA-adding enzyme generates altered specificities. Molecular cell 43 15304219
2023 Imaging in metastatic breast cancer, CT, PET/CT, MRI, WB-DWI, CCA: review and new perspectives. Cancer imaging : the official publication of the International Cancer Imaging Society 41 37254225
2017 Chelant-enhanced washing of CCA-contaminated soil: Coupled with selective dissolution or soil stabilization. The Science of the total environment 40 28903175
1998 The Bacillus subtilis nucleotidyltransferase is a tRNA CCA-adding enzyme. Journal of bacteriology 40 9829937
1998 Participation of the 3'-CCA of tRNA in the binding of catalytic Mg2+ ions by ribonuclease P. Biochemistry 39 9585541
2009 Membrane-less cloth cathode assembly (CCA) for scalable microbial fuel cells. Biosensors & bioelectronics 37 19556120
2015 Carcinogenic risk of chromium, copper and arsenic in CCA-treated wood. Environmental pollution (Barking, Essex : 1987) 35 26275730
2008 tRNA integrity is a prerequisite for rapid CCA addition: implication for quality control. Journal of molecular biology 35 18466919
2000 Unusual synthesis by the Escherichia coli CCA-adding enzyme. RNA (New York, N.Y.) 35 10917598
1997 De novo and DNA primer-mediated initiation of cDNA synthesis by the mauriceville retroplasmid reverse transcriptase involve recognition of a 3' CCA sequence. Journal of molecular biology 34 9268661
2015 The CCA-adding enzyme: A central scrutinizer in tRNA quality control. BioEssays : news and reviews in molecular, cellular and developmental biology 33 26172425
2003 Association of positional and functional candidate genes FGF1, FBN2, and LOX on 5q31 with intracranial aneurysm. Journal of human genetics 33 12750963
2000 CCA initiation boxes without unique promoter elements support in vitro transcription by three viral RNA-dependent RNA polymerases. RNA (New York, N.Y.) 33 10836791
2018 Evaluating a point-of-care circulating cathodic antigen test (POC-CCA) to detect Schistosoma mansoni infections in a low endemic area in north-eastern Brazil. Acta tropica 32 29526480
2010 Phase-II trial of combination treatment of interferon-α, cimetidine, cyclooxygenase-2 inhibitor and renin-angiotensin-system inhibitor (I-CCA therapy) for advanced renal cell carcinoma. Cancer science 32 20973869
2007 In vivo and in vitro investigation of bacterial type B RNase P interaction with tRNA 3'-CCA. Nucleic acids research 32 17355991
1996 The donor substrate site within the peptidyl transferase loop of 23 S rRNA and its putative interactions with the CCA-end of N-blocked aminoacyl-tRNA(Phe). Journal of molecular biology 32 8969299
2021 RelA-SpoT Homolog toxins pyrophosphorylate the CCA end of tRNA to inhibit protein synthesis. Molecular cell 31 34174184
2013 Ocular phenotype of Fbn2-null mice. Investigative ophthalmology & visual science 31 24130178
2015 Fine genetic mapping of target leaf spot resistance gene cca-3 in cucumber, Cucumis sativus L. TAG. Theoretical and applied genetics. Theoretische und angewandte Genetik 30 26385372
2008 2'-5' oligoadenylate synthetase shares active site architecture with the archaeal CCA-adding enzyme. Cellular and molecular life sciences : CMLS 30 18604630
1989 Metastatic ability and differentiative properties of a new cell line of human embryonal rhabdomyosarcoma (CCA). Anticancer research 30 2516717
2022 The fibrillinopathies: New insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2. Human mutation 29 35419902
2002 Poly(C) synthesis by class I and class II CCA-adding enzymes. Biochemistry 29 11926813
2016 Developing multi-cellular tumor spheroid model (MCTS) in the chitosan/collagen/alginate (CCA) fibrous scaffold for anticancer drug screening. Materials science & engineering. C, Materials for biological applications 28 26952417
2014 ELN and FBN2 gene variants as risk factors for two sports-related musculoskeletal injuries. International journal of sports medicine 28 25429546
2010 How the CCA-adding enzyme selects adenine over cytosine at position 76 of tRNA. Science (New York, N.Y.) 28 21071662
2003 Decreased CCA-addition in human mitochondrial tRNAs bearing a pathogenic A4317G or A10044G mutation. The Journal of biological chemistry 28 12621050
2019 LOTTE-seq (Long hairpin oligonucleotide based tRNA high-throughput sequencing): specific selection of tRNAs with 3'-CCA end for high-throughput sequencing. RNA biology 27 31486704
2011 The vitamin D receptor gene bAt (CCA) haplotype impairs the response to pegylated-interferon/ribavirin-based therapy in chronic hepatitis C patients. Antiviral therapy 27 22300961
2008 Role of the CCA bulge of prohead RNA of bacteriophage ø29 in DNA packaging. Journal of molecular biology 27 18778713
2022 Selective Cleavage at CCA Ends and Anticodon Loops of tRNAs by Stress-Induced RNases. Frontiers in molecular biosciences 26 35300117
2014 Decreased tobacco-specific nitrosamines by microbial treatment with Bacillus amyloliquefaciens DA9 during the air-curing process of burley tobacco. Journal of agricultural and food chemistry 26 25514373
2005 Repair of the tRNA-like CCA sequence in a multipartite positive-strand RNA virus. Journal of virology 25 15650168
2001 U2 small nuclear RNA is a substrate for the CCA-adding enzyme (tRNA nucleotidyltransferase). The Journal of biological chemistry 25 11700323
1999 Substrate binding and catalysis by ribonuclease P from cyanobacteria and Escherichia coli are affected differently by the 3' terminal CCA in tRNA precursors. Proceedings of the National Academy of Sciences of the United States of America 25 10359770
2008 Molecular basis for maintenance of fidelity during the CCA-adding reaction by a CCA-adding enzyme. The EMBO journal 24 18583961
2020 Diseases Associated with Defects in tRNA CCA Addition. International journal of molecular sciences 23 32471101
2008 A comparative analysis of two conserved motifs in bacterial poly(A) polymerase and CCA-adding enzyme. Nucleic acids research 22 18682528
2007 Hfq stimulates the activity of the CCA-adding enzyme. BMC molecular biology 22 17949481
1987 An immunomodulating anti-rheumatic drug, lobenzarit disodium (CCA): inhibition of polyclonal B-cell activation and prevention of autoimmune disease in MRL/Mp-lpr/lpr mice. Clinical immunology and immunopathology 22 3500002
2008 Peptidyl-CCA deacylation on the ribosome promoted by induced fit and the O3'-hydroxyl group of A76 of the unacylated A-site tRNA. RNA (New York, N.Y.) 21 18818369
2004 Archaeal CCA-adding enzymes: central role of a highly conserved beta-turn motif in RNA polymerization without translocation. The Journal of biological chemistry 21 15590678
2003 Use of nucleotide analogs by class I and class II CCA-adding enzymes (tRNA nucleotidyltransferase): deciphering the basis for nucleotide selection. RNA (New York, N.Y.) 21 12869708
1995 DNA methylation in mycobacteria: absence of methylation at GATC (Dam) and CCA/TGG (Dcm) sequences. FEMS immunology and medical microbiology 21 8541807
2024 The scaffold of neutrophil extracellular traps promotes CCA progression and modulates angiogenesis via ITGAV/NFκB. Cell communication and signaling : CCS 20 38326837
2016 Evaluation of the CCA Immuno-Chromatographic Test to Diagnose Schistosoma mansoni in Minas Gerais State, Brazil. PLoS neglected tropical diseases 20 26752073
2015 The identity of the discriminator base has an impact on CCA addition. Nucleic acids research 20 25958396
2014 A syndromic form of Pierre Robin sequence is caused by 5q23 deletions encompassing FBN2 and PHAX. European journal of medical genetics 20 25195018
2000 Sulfolobus shibatae CCA-adding enzyme forms a tetramer upon binding two tRNA molecules: A scrunching-shuttling model of CCA specificity. Journal of molecular biology 20 11090289
2022 Diagnosis of Schistosomiasis without a Microscope: Evaluating Circulating Antigen (CCA, CAA) and DNA Detection Methods on Banked Samples of a Community-Based Survey from DR Congo. Tropical medicine and infectious disease 19 36288056
2022 Crenigacestat blocking notch pathway reduces liver fibrosis in the surrounding ecosystem of intrahepatic CCA viaTGF-β inhibition. Journal of experimental & clinical cancer research : CR 19 36443822
2017 Examining tRNA 3'-ends in Escherichia coli: teamwork between CCA-adding enzyme, RNase T, and RNase R. RNA (New York, N.Y.) 19 29180590
2012 Lys53 of ribosomal protein L36AL and the CCA end of a tRNA at the P/E hybrid site are in close proximity on the human ribosome. Chembiochem : a European journal of chemical biology 19 22865768
1991 Peculiarities of recognition of CCA/TGG sequences in DNA by restriction endonucleases MvaI and EcoRII. Journal of molecular recognition : JMR 19 1665978
2020 Meta-analysis of genome-wide association study identifies FBN2 as a novel locus associated with systemic lupus erythematosus in Thai population. Arthritis research & therapy 18 32771030
2016 Impaired activity of CCA-adding enzyme TRNT1 impacts OXPHOS complexes and cellular respiration in SIFD patient-derived fibroblasts. Orphanet journal of rare diseases 17 27317422
2006 A model for C74 addition by CCA-adding enzymes: C74 addition, like C75 and A76 addition, does not involve tRNA translocation. The Journal of biological chemistry 17 16455665
1995 Chromosome condensation activity (CCA) in bisected C57BL/6JxCBA mouse oocytes. Reproduction, fertility, and development 17 8848580
2016 Identification of transcription factors (TFs) and targets involved in the cholangiocarcinoma (CCA) by integrated analysis. Cancer gene therapy 16 27857060
2009 Distinct kinetic determinants for the stepwise CCA addition to tRNA. RNA (New York, N.Y.) 16 19696158
1994 Of mice and Marfan: genetic linkage analyses of the fibrillin genes, Fbn1 and Fbn2, in the mouse genome. Mammalian genome : official journal of the International Mammalian Genome Society 16 7873879
1983 Effect of N-(2-carboxyphenyl)-4-chloroanthranilic acid disodium salt (CCA) on the induction of helper and suppressor T cells in vitro and in vivo. Japanese journal of pharmacology 16 6226816
2020 Diagnostic comparison of stool exam and point-of-care circulating cathodic antigen (POC-CCA) test for schistosomiasis mansoni diagnosis in a high endemicity area in northeastern Brazil. Parasitology 15 33190646
2010 Mode of action of RNase BN/RNase Z on tRNA precursors: RNase BN does not remove the CCA sequence from tRNA. The Journal of biological chemistry 15 20489203
2005 Metal-ion-dependent catalysis and specificity of CCA-adding enzymes: a comparison of two classes. Biochemistry 15 16171400
2023 Revealing the role of necroptosis microenvironment: FCGBP + tumor-associated macrophages drive primary liver cancer differentiation towards cHCC-CCA or iCCA. Apoptosis : an international journal on programmed cell death 14 38017206
2022 Incorporating oral, inhalation and dermal bioaccessibility into human health risk characterization following exposure to Chromated Copper Arsenate (CCA)-contaminated soils. Ecotoxicology and environmental safety 14 38321665