{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T15:42:22Z","timestamp":1785426142099,"version":"3.56.0"},"reference-count":111,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T00:00:00Z","timestamp":1781568000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["INST 211\/1038-1 FUGG"],"award-info":[{"award-number":["INST 211\/1038-1 FUGG"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["INST 211\/744-1 FUGG"],"award-info":[{"award-number":["INST 211\/744-1 FUGG"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"award":["INST 211\/1038-1 FUGG"],"award-info":[{"award-number":["INST 211\/1038-1 FUGG"]}],"id":[{"id":"https:\/\/ror.org\/018mejw64","id-type":"ROR","asserted-by":"publisher"}]},{"award":["INST 211\/744-1 FUGG"],"award-info":[{"award-number":["INST 211\/744-1 FUGG"]}],"id":[{"id":"https:\/\/ror.org\/018mejw64","id-type":"ROR","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The lysosomal enzyme \u03b1-galactosidase A (AGAL) degrades globotriaosylceramide (Gb3). While this enzymatic function in lysosomal metabolism is well characterized, interaction partners and alternative functions are unknown. This study aims to identify new potential AGAL-interacting proteins. AGAL was fused to the mutated biotin ligase BirA from E. coli (TurboID). Expression of the fusion protein was confirmed by Western blot and immunofluorescence, while enzymatic activity was verified by functional assays. In three experimental settings (AGAL wild-type (WT), AGAL missense variant (p.N215S), and the control cell line), TurboID-biotinylated proximal proteins were enriched by streptavidin pull-down and analyzed by mass spectrometry. Gene Ontology (GO) terms were subsequently evaluated to characterize biological functions and localizations of the identified proteins. Selected candidates were co-immunoprecipitated with AGAL to confirm direct interactions. The AGAL-TurboID fusion protein was successfully expressed in AB8\/13 podocytes. Immunofluorescence and enzyme activity assays confirmed the presence and functionality of the fusion protein. Subsequent functional analysis (GO term analysis) showed enrichment of driver terms, including extracellular matrix organization (ECM), multicellular organism development, and protein metabolic process, in the biological process category. The identified top-hit proteins were predominantly involved in the organization of ECM, cell proliferation and cytokinesis, unfolded protein response during endoplasmic reticulum stress, and protein ubiquitination. Co-immunoprecipitation confirmed the interaction between AGAL and the candidate Galectin-3-binding protein (Gal-3BP). Our results suggest that AGAL may play a role in other pathways and\/or the ECM organization beyond its lysosomal function. The confirmed interaction with Gal-3BP can now be functionally investigated in further studies.<\/jats:p>","DOI":"10.3390\/ijms27125437","type":"journal-article","created":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T03:27:21Z","timestamp":1781666841000},"page":"5437","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Identification of Potential Proteins Interacting with \u03b1-Galactosidase A to Analyze the Pathogenesis of Fabry Disease"],"prefix":"10.3390","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-5579-8417","authenticated-orcid":false,"given":"Elise Raphaela","family":"Menke","sequence":"first","affiliation":[{"name":"Internal Medicine D (Nephrology, Hypertension and Rheumatology), and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Eirich","sequence":"additional","affiliation":[{"name":"Institute of Plant Biology and Biotechnology, University of Muenster, 48143 Muenster, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"Finkemeier","sequence":"additional","affiliation":[{"name":"Institute of Plant Biology and Biotechnology, University of Muenster, 48143 Muenster, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malte","family":"Lenders","sequence":"additional","affiliation":[{"name":"Internal Medicine D (Nephrology, Hypertension and Rheumatology), and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva","family":"Brand","sequence":"additional","affiliation":[{"name":"Internal Medicine D (Nephrology, Hypertension and Rheumatology), and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.jmb.2004.01.035","article-title":"The molecular defect leading to Fabry disease: Structure of human alpha-galactosidase","volume":"337","author":"Garman","year":"2004","journal-title":"J. Mol. Biol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1016\/S0140-6736(08)61589-5","article-title":"Fabry\u2019s disease","volume":"372","author":"Zarate","year":"2008","journal-title":"Lancet"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"5974","DOI":"10.2174\/13816128113199990352","article-title":"Anderson-Fabry disease: A multiorgan disease","volume":"19","author":"Tuttolomondo","year":"2013","journal-title":"Curr. Pharm. Des."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1681\/ASN.2019050497","article-title":"Accumulation of Globotriaosylceramide in Podocytes in Fabry Nephropathy Is Associated with Progressive Podocyte Loss","volume":"31","author":"Najafian","year":"2020","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.trsl.2023.02.006","article-title":"Deep next-generation proteomics and network analysis reveal systemic and tissue-specific patterns in Fabry disease","volume":"258","author":"Tebani","year":"2023","journal-title":"Transl. Res."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Fernandes, M., and Husi, H. (2016). Integrative Systems Biology Investigation of Fabry Disease. Diseases, 4.","DOI":"10.3390\/diseases4040035"},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Jehn, U., Bayraktar, S., Pollmann, S., van Marck, V., Weide, T., Pavenst\u00e4dt, H., Brand, E., and Lenders, M. (2021). \u03b1-Galactosidase a Deficiency in Fabry Disease Leads to Extensive Dysregulated Cellular Signaling Pathways in Human Podocytes. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms222111339"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/0014-5793(96)00863-0","article-title":"Only sphingolipid activator protein B (SAP-B or saposin B) stimulates the degradation of globotriaosylceramide by recombinant human lysosomal alpha-galactosidase in a detergent-free liposomal system","volume":"393","author":"Kase","year":"1996","journal-title":"FEBS Lett."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Sawyer, T.K., Aral, E., Staros, J.V., Bobst, C.E., and Garman, S.C. (2024). Human Saposin B Ligand Binding and Presentation to \u03b1-Galactosidase A. bioRxiv, bioRxiv:04.04.584535.","DOI":"10.1101\/2024.04.04.584535"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/j.bbamcr.2008.10.016","article-title":"Sorting of lysosomal proteins","volume":"1793","author":"Braulke","year":"2009","journal-title":"Biochim Biophys. Acta"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Prabakaran, T., Nielsen, R., Satchell, S.C., Mathieson, P.W., Feldt-Rasmussen, U., S\u00f8rensen, S.S., and Christensen, E.I. (2012). Mannose 6-phosphate receptor and sortilin mediated endocytosis of \u03b1-galactosidase A in kidney endothelial cells. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0039975"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"48","DOI":"10.4161\/cam.22826","article-title":"The laminin family","volume":"7","author":"Aumailley","year":"2013","journal-title":"Cell Adh Migr."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"4195","DOI":"10.1242\/jcs.023820","article-title":"The extracellular matrix at a glance","volume":"123","author":"Frantz","year":"2010","journal-title":"J. Cell Sci."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"4837","DOI":"10.1093\/hmg\/ddq414","article-title":"FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy","volume":"19","author":"Fassone","year":"2010","journal-title":"Hum. Mol. Genet"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"7509","DOI":"10.1038\/s41380-021-01187-x","article-title":"Cerebellin-2 regulates a serotonergic dorsal raphe circuit that controls compulsive behaviors","volume":"26","author":"Seigneur","year":"2021","journal-title":"Mol. Psychiatry"},{"key":"ref_16","unstructured":"(2026, June 07). The Human Protein Atlas: CBLN2. Available online: https:\/\/www.proteinatlas.org\/ENSG00000141668-CBLN2."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1074\/jbc.274.2.781","article-title":"An evolutionarily conserved family of Hsp70\/Hsc70 molecular chaperone regulators","volume":"274","author":"Takayama","year":"1999","journal-title":"J. Biol. Chem."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1074\/jbc.M113.521997","article-title":"Binding of human nucleotide exchange factors to heat shock protein 70 (Hsp70) generates functionally distinct complexes in vitro","volume":"289","author":"Rauch","year":"2014","journal-title":"J. Biol. Chem."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Lumbroso, D., Soboh, S., Maimon, A., Schif-Zuck, S., Ariel, A., and Burstyn-Cohen, T. (2018). Macrophage-Derived Protein S Facilitates Apoptotic Polymorphonuclear Cell Clearance by Resolution Phase Macrophages and Supports Their Reprogramming. Front. Immunol., 9.","DOI":"10.3389\/fimmu.2018.00358"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"V\u00e1ncza, L., Kar\u00e1szi, K., P\u00e9terfia, B., Turi\u00e1k, L., Dezs\u0151, K., Sebesty\u00e9n, A., Reszegi, A., Pet\u0151v\u00e1ri, G., Kiss, A., and Schaff, Z. (2022). SPOCK1 Promotes the Development of Hepatocellular Carcinoma. Front. Oncol., 12.","DOI":"10.3389\/fonc.2022.819883"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/S0074-7696(04)36003-1","article-title":"Testican-1: A differentially expressed proteoglycan with protease inhibiting activities","volume":"236","author":"Edgell","year":"2004","journal-title":"Int. Rev. Cytol."},{"key":"ref_22","first-page":"289","article-title":"DDX10 promotes AIM2-inflammasome activation by maintaining AIM2 protein stability","volume":"36","author":"Zhao","year":"2020","journal-title":"Chin. J. Cell Mol. Immunol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1042\/bj3320789","article-title":"Human alpha-galactosidase A: Glycosylation site 3 is essential for enzyme solubility","volume":"332","author":"Ioannou","year":"1998","journal-title":"Biochem. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"105733","DOI":"10.1016\/j.ebiom.2025.105733","article-title":"Impact of ER stress and the unfolded protein response on Fabry disease","volume":"115","author":"Lenders","year":"2025","journal-title":"EBioMedicine"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1007\/s10545-006-0360-6","article-title":"Extracellular matrix turnover and disease severity in Anderson-Fabry disease","volume":"30","author":"Shah","year":"2007","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Loso, J., Lund, N., Avanesov, M., Muschol, N., Lezius, S., Cordts, K., Schwedhelm, E., and Patten, M. (2018). Serum Biomarkers of Endothelial Dysfunction in Fabry Associated Cardiomyopathy. Front. Cardiovasc. Med., 5.","DOI":"10.3389\/fcvm.2018.00108"},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Pollmann, S., Scharnetzki, D., Manikowski, D., Lenders, M., and Brand, E. (2021). Endothelial Dysfunction in Fabry Disease Is Related to Glycocalyx Degradation. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.789142"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1016\/j.kint.2026.01.004","article-title":"Heat shock protein 70s are modifiers of endothelial function in Fabry disease","volume":"109","author":"Verma","year":"2026","journal-title":"Kidney Int."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1606","DOI":"10.1093\/emboj\/17.6.1606","article-title":"Mac-2 binding protein is a cell-adhesive protein of the extracellular matrix which self-assembles into ring-like structures and binds beta1 integrins, collagens and fibronectin","volume":"17","author":"Sasaki","year":"1998","journal-title":"EMBO J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"18401","DOI":"10.1016\/S0021-9258(17)32322-0","article-title":"The secreted tumor-associated antigen 90K is a potent immune stimulator","volume":"269","author":"Ullrich","year":"1994","journal-title":"J. Biol. Chem."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1038\/bjc.1994.29","article-title":"Prognostic value of a novel circulating serum 90K antigen in breast cancer","volume":"69","author":"Iacobelli","year":"1994","journal-title":"Br. J. Cancer"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"100793","DOI":"10.1016\/j.mcpro.2024.100793","article-title":"Galectin-3-Binding Protein Inhibits Extracellular Heparan 6-O-Endosulfatase Sulf-2","volume":"23","author":"Panigrahi","year":"2024","journal-title":"Mol. Cell Proteom."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1002\/mgg3.389","article-title":"Phenotypic characteristics of the p.Asn215Ser (p.N215S) GLA mutation in male and female patients with Fabry disease: A multicenter Fabry Registry study","volume":"6","author":"Germain","year":"2018","journal-title":"Mol. Genet. Genom. Med."},{"key":"ref_34","doi-asserted-by":"crossref","unstructured":"Isinelli, G., Scacci, G., Capocchia, A., Emiliani, C., Mecucci, C., La Starza, R., and Di Giacomo, D. (2026). DDX10 RNA Helicase: Structure, Function, and Oncogenic Roles Across Solid and Hematologic Tumors. Genes, 17.","DOI":"10.3390\/genes17020138"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1038\/nbt.4201","article-title":"Efficient proximity labeling in living cells and organisms with TurboID","volume":"36","author":"Branon","year":"2018","journal-title":"Nat. Biotechnol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1397","DOI":"10.1074\/mcp.M113.034108","article-title":"The Vac14-interaction network is linked to regulators of the endolysosomal and autophagic pathway","volume":"13","author":"Schulze","year":"2014","journal-title":"Mol. Cell Proteom."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3971","DOI":"10.1038\/s41596-020-0399-0","article-title":"Proximity labeling in mammalian cells with TurboID and split-TurboID","volume":"15","author":"Cho","year":"2020","journal-title":"Nat. Protoc."},{"key":"ref_38","first-page":"157","article-title":"Fabry\u2019s disease: Enzymatic diagnosis of hemizygotes and heterozygotes. Alpha-galactosidase activities in plasma, serum, urine, and leukocytes","volume":"81","author":"Desnick","year":"1973","journal-title":"J. Lab Clin. Med."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/0009-8981(81)90384-3","article-title":"Differential assay for lysosomal alpha-galactosidases in human tissues and its application to Fabry\u2019s disease","volume":"112","author":"Mayes","year":"1981","journal-title":"Clin. Chim. Acta"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1038\/s41596-018-0082-x","article-title":"Single-pot, solid-phase-enhanced sample preparation for proteomics experiments","volume":"14","author":"Hughes","year":"2019","journal-title":"Nat. Protoc."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1038\/nmeth.4256","article-title":"MSFragger: Ultrafast and comprehensive peptide identification in mass spectrometry-based proteomics","volume":"14","author":"Kong","year":"2017","journal-title":"Nat. Methods"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"100077","DOI":"10.1016\/j.mcpro.2021.100077","article-title":"IonQuant Enables Accurate and Sensitive Label-Free Quantification With FDR-Controlled Match-Between-Runs","volume":"20","author":"Yu","year":"2021","journal-title":"Mol. Cell Proteom."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"The UniProt Consortium (2025). UniProt: The Universal Protein Knowledgebase in 2025. Nucleic Acids Res., 53, D609\u2013D617.","DOI":"10.1093\/nar\/gkae1010"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1038\/s41592-020-0912-y","article-title":"Philosopher: A versatile toolkit for shotgun proteomics data analysis","volume":"17","author":"Haynes","year":"2020","journal-title":"Nat. Methods"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2513","DOI":"10.1074\/mcp.M113.031591","article-title":"Accurate proteome-wide label-free quantification by delayed normalization and maximal peptide ratio extraction, termed MaxLFQ","volume":"13","author":"Cox","year":"2014","journal-title":"Mol. Cell Proteom."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/978-1-0716-1967-4_6","article-title":"Left-Censored Missing Value Imputation Approach for MS-Based Proteomics Data with GSimp","volume":"2426","author":"Wei","year":"2021","journal-title":"Methods Mol. Biol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1214\/16-AOAS920","article-title":"Robust hyperparameter estimation protects against hypervariable genes and improves power to detect differential expression","volume":"10","author":"Phipson","year":"2016","journal-title":"Ann. Appl. Stat."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/gkv007","article-title":"limma powers differential expression analyses for RNA-sequencing and microarray studies","volume":"43","author":"Ritchie","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1038\/s41467-021-27722-6","article-title":"Proximity labeling identifies a repertoire of site-specific R-loop modulators","volume":"13","author":"Yan","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"jcs261952","DOI":"10.1242\/jcs.261952","article-title":"When less is more\u2014A fast TurboID knock-in approach for high-sensitivity endogenous interactome mapping","volume":"137","author":"Stockhammer","year":"2024","journal-title":"J. Cell Sci."},{"key":"ref_51","first-page":"6003","article-title":"The absence of NUDC in cone photoreceptors causes protein mislocalization and disrupts the scotopic electroretinographic b-wave","volume":"66","author":"Gross","year":"2025","journal-title":"Investig. Ophthalmol. Vis. Sci."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1038\/cr.2015.152","article-title":"NudC regulates actin dynamics and ciliogenesis by stabilizing cofilin 1","volume":"26","author":"Zhang","year":"2016","journal-title":"Cell Res."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"583507","DOI":"10.1155\/2014\/583507","article-title":"Motor training in degenerative spinocerebellar disease: Ataxia-specific improvements by intensive physiotherapy and exergames","volume":"2014","author":"Synofzik","year":"2014","journal-title":"Biomed. Res. Int."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"15558","DOI":"10.1074\/jbc.M212074200","article-title":"P58IPK, a novel endoplasmic reticulum stress-inducible protein and potential negative regulator of eIF2alpha signaling","volume":"278","author":"Martindale","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"15920","DOI":"10.1073\/pnas.252341799","article-title":"Control of PERK eIF2alpha kinase activity by the endoplasmic reticulum stress-induced molecular chaperone P58IPK","volume":"99","author":"Yan","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"33066","DOI":"10.1074\/jbc.M505023200","article-title":"Tissue-specific expression and dimerization of the endoplasmic reticulum oxidoreductase Ero1beta","volume":"280","author":"Gubbens","year":"2005","journal-title":"J. Biol. Chem."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1093\/emboj\/21.4.835","article-title":"ERp44, a novel endoplasmic reticulum folding assistant of the thioredoxin family","volume":"21","author":"Anelli","year":"2002","journal-title":"EMBO J."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"591","DOI":"10.34067\/KID.0000000000000094","article-title":"Lrpap1 (RAP) Inhibits Proximal Tubule Clathrin Mediated and Clathrin Independent Endocytosis, Ameliorating Renal Aminoglycoside Nephrotoxicity","volume":"4","author":"Wagner","year":"2023","journal-title":"Kidney360"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"2269","DOI":"10.1002\/j.1460-2075.1995.tb07221.x","article-title":"39 kDa receptor-associated protein is an ER resident protein and molecular chaperone for LDL receptor-related protein","volume":"14","author":"Bu","year":"1995","journal-title":"EMBO J."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.ajhg.2013.06.002","article-title":"Mutations in LRPAP1 are associated with severe myopia in humans","volume":"93","author":"Aldahmesh","year":"2013","journal-title":"Am. J. Hum. Genet"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1517\/14728222.2010.504718","article-title":"Sulf-2: An extracellular modulator of cell signaling and a cancer target candidate","volume":"14","author":"Rosen","year":"2010","journal-title":"Expert. Opin. Ther. Targets"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1038\/s41389-021-00374-3","article-title":"CCT8 recovers WTp53-suppressed cell cycle evolution and EMT to promote colorectal cancer progression","volume":"10","author":"Liao","year":"2021","journal-title":"Oncogenesis"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1038\/ni1066","article-title":"Triad3A, an E3 ubiquitin-protein ligase regulating Toll-like receptors","volume":"5","author":"Chuang","year":"2004","journal-title":"Nat. Immunol."},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Nakhaei, P., Mesplede, T., Solis, M., Sun, Q., Zhao, T., Yang, L., Chuang, T.-H., Ware, C.F., Lin, R., and Hiscott, J. (2009). The E3 ubiquitin ligase Triad3A negatively regulates the RIG-I\/MAVS signaling pathway by targeting TRAF3 for degradation. PLoS Pathog., 5.","DOI":"10.1371\/journal.ppat.1000650"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1501","DOI":"10.1038\/s41467-018-03942-1","article-title":"RPAP3 provides a flexible scaffold for coupling HSP90 to the human R2TP co-chaperone complex","volume":"9","author":"Martino","year":"2018","journal-title":"Nat. Commun."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/978-3-030-00737-9_4","article-title":"The Multiple Functions of the PAQosome: An R2TP- and URI1 Prefoldin-Based Chaperone Complex","volume":"1106","author":"Lynham","year":"2018","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1242\/dev.01037","article-title":"Collagen IV is essential for basement membrane stability but dispensable for initiation of its assembly during early development","volume":"131","author":"Brachvogel","year":"2004","journal-title":"Development"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"F1680","DOI":"10.1152\/ajprenal.00266.2019","article-title":"Differential expression of parietal epithelial cell and podocyte extracellular matrix proteins in focal segmental glomerulosclerosis and diabetic nephropathy","volume":"317","author":"Chan","year":"2019","journal-title":"Am. J. Physiol. Ren. Physiol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1038\/sj.cdd.4401502","article-title":"A highly conserved proapoptotic gene, IKIP, located next to the APAF1 gene locus, is regulated by p53","volume":"11","author":"Schmid","year":"2004","journal-title":"Cell Death Differ."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"418","DOI":"10.4049\/jimmunol.1900626","article-title":"IKIP Negatively Regulates NF-\u03baB Activation and Inflammation through Inhibition of IKK\u03b1\/\u03b2 Phosphorylation","volume":"204","author":"Wu","year":"2020","journal-title":"J. Immunol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1016\/j.matbio.2018.04.008","article-title":"Alport syndrome and Pierson syndrome: Diseases of the glomerular basement membrane","volume":"71","author":"Funk","year":"2018","journal-title":"Matrix Biol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"e145908","DOI":"10.1172\/jci.insight.145908","article-title":"Laminin \u03b22 variants associated with isolated nephropathy that impact matrix regulation","volume":"6","author":"Kikkawa","year":"2021","journal-title":"JCI Insight"},{"key":"ref_73","unstructured":"White, M. (2015). Linking Fibrosis and Cancer through the Differentiation of Fibrocytes. [Doctoral Dissertation, Texas A&M University]."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"E10687","DOI":"10.1073\/pnas.1705682114","article-title":"EB1-binding-myomegalin protein complex promotes centrosomal microtubules functions","volume":"114","author":"Bouguenina","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"3457","DOI":"10.1242\/jcs.115.17.3457","article-title":"Secretion of human latent TGF-beta-binding protein-3 (LTBP-3) is dependent on co-expression of TGF-beta","volume":"115","author":"Penttinen","year":"2002","journal-title":"J. Cell Sci."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"2691","DOI":"10.1091\/mbc.11.8.2691","article-title":"Specific sequence motif of 8-Cys repeats of TGF-beta binding proteins, LTBPs, creates a hydrophobic interaction surface for binding of small latent TGF-beta","volume":"11","author":"Saharinen","year":"2000","journal-title":"Mol. Biol. Cell"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/S0955-0674(99)00081-2","article-title":"Smads as transcriptional co-modulators","volume":"12","author":"Attisano","year":"2000","journal-title":"Curr. Opin. Cell Biol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"27120","DOI":"10.18632\/oncotarget.15449","article-title":"Gambogic acid suppresses cancer invasion and migration by inhibiting TGF\u03b21-induced epithelial-to-mesenchymal transition","volume":"8","author":"Zhao","year":"2017","journal-title":"Oncotarget"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1042\/bj20030532","article-title":"Characterization of SMOC-2, a modular extracellular calcium-binding protein","volume":"373","author":"Vannahme","year":"2003","journal-title":"Biochem. J."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1016\/j.biocel.2011.12.021","article-title":"Diverse biological functions of the SPARC family of proteins","volume":"44","author":"Bradshaw","year":"2012","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1093\/cvr\/cvab107","article-title":"SPARC-related modular calcium binding 1 regulates aortic valve calcification by disrupting BMPR-II\/p-p38 signalling","volume":"118","author":"Wang","year":"2022","journal-title":"Cardiovasc. Res."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1016\/j.bbrc.2019.01.114","article-title":"DDX10 overexpression predicts worse prognosis in osteosarcoma and its deletion prohibits cell activities modulated by MAPK pathway","volume":"510","author":"Shi","year":"2019","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"697","DOI":"10.34133\/research.0697","article-title":"DDX10 Exacerbates Exosomal PD-L1-Dependent T Cell Exhaustion via Phase Separation of Rab27b in Oral Squamous Cell Carcinoma","volume":"8","author":"Li","year":"2025","journal-title":"Research"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"2257","DOI":"10.1080\/15548627.2023.2179844","article-title":"Porcine reproductive and respiratory syndrome virus degrades DDX10 via SQSTM1\/p62-dependent selective autophagy to antagonize its antiviral activity","volume":"19","author":"Li","year":"2023","journal-title":"Autophagy"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1873","DOI":"10.1111\/1759-7714.13976","article-title":"DDX10 promotes human lung carcinoma proliferation by U3 small nucleolar ribonucleoprotein IMP4","volume":"12","author":"Liu","year":"2021","journal-title":"Thorac. Cancer"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1186\/s12935-022-02478-1","article-title":"DDX10 promotes the proliferation and metastasis of colorectal cancer cells via splicing RPL35","volume":"22","author":"Zhou","year":"2022","journal-title":"Cancer Cell Int."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1111\/j.1432-1033.1988.tb14321.x","article-title":"The arrangement of intra- and intermolecular disulfide bonds in the carboxyterminal, non-collagenous aggregation and cross-linking domain of basement-membrane type IV collagen","volume":"176","author":"Siebold","year":"1988","journal-title":"Eur. J. Biochem."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1021\/acs.jproteome.5b00767","article-title":"Comprehensive Characterization of Glycosylation and Hydroxylation of Basement Membrane Collagen IV by High-Resolution Mass Spectrometry","volume":"15","author":"Basak","year":"2016","journal-title":"J. Proteome Res."},{"key":"ref_89","first-page":"2520","article-title":"Anti-angiogenic cues from vascular basement membrane collagen","volume":"60","author":"Colorado","year":"2000","journal-title":"Cancer Res."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1016\/j.cell.2008.12.025","article-title":"TANGO1 facilitates cargo loading at endoplasmic reticulum exit sites","volume":"136","author":"Saito","year":"2009","journal-title":"Cell"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"4353","DOI":"10.1158\/0008-5472.CAN-04-3536","article-title":"Canstatin acts on endothelial and tumor cells via mitochondrial damage initiated through interaction with alphavbeta3 and alphavbeta5 integrins","volume":"65","author":"Magnon","year":"2005","journal-title":"Cancer Res."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1002\/humu.21401","article-title":"Mapping structural landmarks, ligand binding sites, and missense mutations to the collagen IV heterotrimers predicts major functional domains, novel interactions, and variation in phenotypes in inherited diseases affecting basement membranes","volume":"32","author":"Pedchenko","year":"2011","journal-title":"Hum. Mutat."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1074\/jbc.275.2.1209","article-title":"Canstatin, a novel matrix-derived inhibitor of angiogenesis and tumor growth","volume":"275","author":"Kamphaus","year":"2000","journal-title":"J. Biol. Chem."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1083\/jcb.152.6.1233","article-title":"Oligomerization-dependent regulation of motility and morphogenesis by the collagen XVIII NC1\/endostatin domain","volume":"152","author":"Kuo","year":"2001","journal-title":"J. Cell Biol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"27872","DOI":"10.1074\/jbc.M202771200","article-title":"Endostatin blocks vascular endothelial growth factor-mediated signaling via direct interaction with KDR\/Flk-1","volume":"277","author":"Kim","year":"2002","journal-title":"J. Biol. Chem."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/S0092-8674(00)81848-6","article-title":"Endostatin: An endogenous inhibitor of angiogenesis and tumor growth","volume":"88","author":"Boehm","year":"1997","journal-title":"Cell"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"4201","DOI":"10.1242\/jcs.00106","article-title":"New functional roles for non-collagenous domains of basement membrane collagens","volume":"115","author":"Ortega","year":"2002","journal-title":"J. Cell Sci."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/S1097-2765(01)00225-8","article-title":"Cell surface glypicans are low-affinity endostatin receptors","volume":"7","author":"Karumanchi","year":"2001","journal-title":"Mol. Cell"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"1024","DOI":"10.1073\/pnas.98.3.1024","article-title":"Interaction of endostatin with integrins implicated in angiogenesis","volume":"98","author":"Rehn","year":"2001","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"11087","DOI":"10.1074\/jbc.RA119.007806","article-title":"Differential cleavage of lysyl oxidase by the metalloproteinases BMP1 and ADAMTS2\/14 regulates collagen binding through a tyrosine sulfate domain","volume":"294","author":"Paradela","year":"2019","journal-title":"J. Biol. Chem."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1074\/jbc.M115.681775","article-title":"Characterization of Microfibrillar-associated Protein 4 (MFAP4) as a Tropoelastin- and Fibrillin-binding Protein Involved in Elastic Fiber Formation","volume":"291","author":"Pilecki","year":"2016","journal-title":"J. Biol. Chem."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1038\/70302","article-title":"Melanoma chondroitin sulphate proteoglycan regulates cell spreading through Cdc42, Ack-1 and p130cas","volume":"1","author":"Eisenmann","year":"1999","journal-title":"Nat. Cell Biol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"26110","DOI":"10.1074\/jbc.271.42.26110","article-title":"Binding of the NG2 proteoglycan to type VI collagen and other extracellular matrix molecules","volume":"271","author":"Burg","year":"1996","journal-title":"J. Biol. Chem."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1002\/1097-4644(20001101)79:2<213::AID-JCB50>3.0.CO;2-G","article-title":"The multi-PDZ domain protein MUPP1 is a cytoplasmic ligand for the membrane-spanning proteoglycan NG2","volume":"79","author":"Barritt","year":"2000","journal-title":"J. Cell. Biochem."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"3580","DOI":"10.1091\/mbc.e04-03-0236","article-title":"NG2 proteoglycan promotes endothelial cell motility and angiogenesis via engagement of galectin-3 and alpha3beta1 integrin","volume":"15","author":"Fukushi","year":"2004","journal-title":"Mol. Biol. Cell"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"16831","DOI":"10.1074\/jbc.274.24.16831","article-title":"High-affinity binding of basic fibroblast growth factor and platelet-derived growth factor-AA to the core protein of the NG2 proteoglycan","volume":"274","author":"Goretzki","year":"1999","journal-title":"J. Biol. Chem."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"28625","DOI":"10.1074\/jbc.M002290200","article-title":"Binding of the NG2 proteoglycan to kringle domains modulates the functional properties of angiostatin and plasmin(ogen)","volume":"275","author":"Goretzki","year":"2000","journal-title":"J. Biol. Chem."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1111\/j.1755-148X.2011.00929.x","article-title":"CSPG4, a potential therapeutic target, facilitates malignant progression of melanoma","volume":"24","author":"Price","year":"2011","journal-title":"Pigment Cell Melanoma Res."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"15283","DOI":"10.1038\/s41598-019-51689-6","article-title":"Integrin \u03b111 cytoplasmic tail is required for FAK activation to initiate 3D cell invasion and ERK-mediated cell proliferation","volume":"9","author":"Erusappan","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"4609","DOI":"10.1172\/JCI125890","article-title":"Stromal integrin \u03b111 regulates PDGFR-\u03b2 signaling and promotes breast cancer progression","volume":"129","author":"Primac","year":"2019","journal-title":"J. Clin. Investig."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1083\/jcb.200509019","article-title":"Small GTPase Rab21 regulates cell adhesion and controls endosomal traffic of beta1-integrins","volume":"173","author":"Pellinen","year":"2006","journal-title":"J. Cell Biol."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/27\/12\/5437\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T04:13:39Z","timestamp":1782188019000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/27\/12\/5437"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,16]]},"references-count":111,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2026,6]]}},"alternative-id":["ijms27125437"],"URL":"https:\/\/doi.org\/10.3390\/ijms27125437","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,16]]}}}