{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T05:26:32Z","timestamp":1784697992449,"version":"3.55.0"},"reference-count":50,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2017,8,1]],"date-time":"2017-08-01T00:00:00Z","timestamp":1501545600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,12,13]],"date-time":"2019-12-13T00:00:00Z","timestamp":1576195200000},"content-version":"vor","delay-in-days":864,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","journals.elsevierhealth.com","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["The American Journal of Pathology"],"published-print":{"date-parts":[[2017,8]]},"DOI":"10.1016\/j.ajpath.2017.04.014","type":"journal-article","created":{"date-parts":[[2017,6,16]],"date-time":"2017-06-16T15:15:20Z","timestamp":1497626120000},"page":"1750-1762","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":38,"title":["Cathepsin K in Lymphangioleiomyomatosis"],"prefix":"10.1016","volume":"187","author":[{"given":"Arundhati","family":"Dongre","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debbie","family":"Clements","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew J.","family":"Fisher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon R.","family":"Johnson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ajpath.2017.04.014_bib1","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1183\/09031936.06.00113303","article-title":"Lymphangioleiomyomatosis","volume":"27","author":"Johnson","year":"2006","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ajpath.2017.04.014_bib2","doi-asserted-by":"crossref","first-page":"3807","DOI":"10.1172\/JCI58709","article-title":"Lymphangioleiomyomatosis\u2014a wolf in sheep's clothing","volume":"122","author":"Henske","year":"2012","journal-title":"J Clin Invest"},{"key":"10.1016\/j.ajpath.2017.04.014_bib3","doi-asserted-by":"crossref","first-page":"30958","DOI":"10.1074\/jbc.M202678200","article-title":"Tuberin regulates p70 S6 kinase activation and ribosomal protein S6 phosphorylation. A role for the TSC2 tumor suppressor gene in pulmonary lymphangioleiomyomatosis (LAM)","volume":"277","author":"Goncharova","year":"2002","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ajpath.2017.04.014_bib4","first-page":"224","article-title":"Pulmonary LAM: TSC2 regulates cell migration","volume":"169","author":"Goncharova","year":"2004","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib5","doi-asserted-by":"crossref","first-page":"12455","DOI":"10.1073\/pnas.1104361108","article-title":"Tumorigenesis in tuberous sclerosis complex is autophagy and p62\/sequestosome 1 (SQSTM1)-dependent","volume":"108","author":"Parkhitko","year":"2011","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.ajpath.2017.04.014_bib6","doi-asserted-by":"crossref","first-page":"1236566","DOI":"10.1126\/science.1236566","article-title":"mTORC1 phosphorylation sites encode their sensitivity to starvation and rapamycin","volume":"341","author":"Kang","year":"2013","journal-title":"Science"},{"key":"10.1016\/j.ajpath.2017.04.014_bib7","doi-asserted-by":"crossref","first-page":"L690","DOI":"10.1152\/ajplung.00311.2003","article-title":"The LAM cell: what is it, where does it come from, and why does it grow?","volume":"286","author":"Finlay","year":"2004","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib8","first-page":"28","article-title":"Tumors characterized by the presence of HMB45-positive perivascular epithelioid cell (PEC)\u2014a novel entity in surgical pathology","volume":"3","author":"Pea","year":"1997","journal-title":"Electron J Pathol Histologic Examination"},{"key":"10.1016\/j.ajpath.2017.04.014_bib9","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1164\/ajrccm.187.6.663","article-title":"Exonic mutations of TSC2\/TSC1 are common but not seen in all sporadic pulmonary lymphangioleiomyomatosis","volume":"187","author":"Badri","year":"2013","journal-title":"Am\u00a0J\u00a0Respir Crit Care Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib10","doi-asserted-by":"crossref","first-page":"e0126025","DOI":"10.1371\/journal.pone.0126025","article-title":"Wild type mesenchymal cells contribute to the lung pathology of lymphangioleiomyomatosis","volume":"10","author":"Clements","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.ajpath.2017.04.014_bib11","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1164\/rccm.2010014","article-title":"Basic fibroblast growth factor and its receptors in idiopathic pulmonary fibrosis and lymphangioleiomyomatosis","volume":"166","author":"Inoue","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib12","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1165\/rcmb.2014-0224OC","article-title":"Surfactant dysfunction and lung inflammation in the female mouse model of lymphangioleiomyomatosis","volume":"53","author":"Atochina-Vasserman","year":"2014","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib13","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1152\/physrev.00022.2006","article-title":"Matrix metalloproteinases in lung: multiple, multifarious, and multifaceted","volume":"87","author":"Greenlee","year":"2007","journal-title":"Physiol Rev"},{"key":"10.1016\/j.ajpath.2017.04.014_bib14","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1016\/S0046-8177(97)90061-7","article-title":"Immunohistochemical study of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) in pulmonary lymphangioleiomyomatosis (LAM)","volume":"28","author":"Hayashi","year":"1997","journal-title":"Hum Pathol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib15","doi-asserted-by":"crossref","first-page":"267","DOI":"10.5858\/2000-124-0267-RFAOMM","article-title":"Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis","volume":"124","author":"Matsui","year":"2000","journal-title":"Arch Pathol Lab Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib16","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1016\/S0002-9440(10)63716-9","article-title":"Role of discoidin domain receptors 1 and 2 in human smooth muscle cell-mediated collagen remodeling: potential implications in atherosclerosis and lymphangioleiomyomatosis","volume":"164","author":"Ferri","year":"2004","journal-title":"Am J Pathol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib17","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1165\/rcmb.2009-0050OC","article-title":"Rapamycin-insensitive up-regulation of MMP2 and other genes in TSC2-deficient LAM-like cells","volume":"42","author":"Lee","year":"2010","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib18","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1186\/1465-9921-13-34","article-title":"Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis","volume":"13","author":"Chang","year":"2012","journal-title":"Respir Res"},{"key":"10.1016\/j.ajpath.2017.04.014_bib19","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1016\/j.rmed.2008.07.017","article-title":"Matrix metalloproteinases in blood from patients with LAM","volume":"103","author":"Odajima","year":"2009","journal-title":"Respir Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib20","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.1183\/09031936.00167413","article-title":"A two year randomised placebo controlled trial of doxycycline for lymphangioleiomyomatosis","volume":"43","author":"Chang","year":"2014","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ajpath.2017.04.014_bib21","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1165\/rcmb.2004-0289OC","article-title":"Imbalanced Plasminogen system in lymphangioleiomyomatosis: potential role of serum response factor","volume":"32","author":"Zhe","year":"2005","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib22","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1038\/modpathol.2008.189","article-title":"Cathepsin-k expression in pulmonary lymphangioleiomyomatosis","volume":"22","author":"Chilosi","year":"2009","journal-title":"Mod Pathol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib23","doi-asserted-by":"crossref","first-page":"33","DOI":"10.5858\/2008-0542-RAR1.1","article-title":"Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors","volume":"134","author":"Martignoni","year":"2010","journal-title":"Arch Pathol Lab Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib24","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1016\/j.bbrc.2009.01.139","article-title":"Cathepsin K: a therapeutic target for bone diseases","volume":"380","author":"Zhao","year":"2009","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1016\/j.ajpath.2017.04.014_bib25","doi-asserted-by":"crossref","first-page":"R84","DOI":"10.1186\/bcr3199","article-title":"CD44 enhances invasion of basal-like breast cancer cells by upregulating serine protease and collagen-degrading enzymatic expression and activity","volume":"14","author":"Montgomery","year":"2012","journal-title":"Breast Cancer Res"},{"key":"10.1016\/j.ajpath.2017.04.014_bib26","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.bbapap.2011.10.002","article-title":"Cysteine cathepsins: from structure, function and regulation to new frontiers","volume":"1824","author":"Turk","year":"2012","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.ajpath.2017.04.014_bib27","doi-asserted-by":"crossref","first-page":"84","DOI":"10.18632\/oncotarget.422","article-title":"Recent developments in targeting carbonic anhydrase IX for cancer therapeutics","volume":"3","author":"McDonald","year":"2012","journal-title":"Oncotarget"},{"key":"10.1016\/j.ajpath.2017.04.014_bib28","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.mam.2012.10.008","article-title":"The SLC4 family of bicarbonate transporters","volume":"34","author":"Romero","year":"2013","journal-title":"Mol Aspects Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib29","doi-asserted-by":"crossref","first-page":"L694","DOI":"10.1152\/ajplung.00204.2003","article-title":"Estradiol and tamoxifen stimulate LAM-associated angiomyolipoma cell growth and activate both genomic and nongenomic signaling pathways","volume":"286","author":"Yu","year":"2004","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.ajpath.2017.04.014_bib30","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1172\/JCI7319","article-title":"Tsc2+\/\u2013 mice develop tumors in multiple sites that express gelsolin and are influenced by genetic background","volume":"104","author":"Onda","year":"1999","journal-title":"J Clin Invest"},{"key":"10.1016\/j.ajpath.2017.04.014_bib31","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1006\/meth.2001.1262","article-title":"Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta C(T)) Method","volume":"25","author":"Livak","year":"2001","journal-title":"Methods"},{"key":"10.1016\/j.ajpath.2017.04.014_bib32","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1016\/j.bone.2006.11.028","article-title":"Membrane-bound carbonic anhydrases in osteoclasts","volume":"40","author":"Riihonen","year":"2007","journal-title":"Bone"},{"key":"10.1016\/j.ajpath.2017.04.014_bib33","doi-asserted-by":"crossref","first-page":"13800","DOI":"10.1074\/jbc.M115.638395","article-title":"Cysteine cathepsins activate ELR chemokines and inactivate non-ELR chemokines","volume":"290","author":"Repnik","year":"2015","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ajpath.2017.04.014_bib34","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1016\/j.molmed.2006.07.008","article-title":"MITF: master regulator of melanocyte development and melanoma oncogene","volume":"12","author":"Levy","year":"2006","journal-title":"Trends Mol Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib35","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1016\/j.bone.2004.07.013","article-title":"Everolimus suppresses cancellous bone loss, bone resorption, and cathepsin K expression by osteoclasts","volume":"35","author":"Kneissel","year":"2004","journal-title":"Bone"},{"key":"10.1016\/j.ajpath.2017.04.014_bib36","doi-asserted-by":"crossref","first-page":"2126","DOI":"10.1074\/jbc.271.4.2126","article-title":"Human cathepsin O2, a matrix protein-degrading cysteine protease expressed in osteoclasts. Functional expression of human cathepsin O2 in Spodoptera frugiperda and characterization of the enzyme","volume":"271","author":"Bromme","year":"1996","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ajpath.2017.04.014_bib37","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1016\/j.biochi.2007.08.011","article-title":"Biochemical properties and regulation of cathepsin K activity","volume":"90","author":"Lecaille","year":"2008","journal-title":"Biochimie"},{"key":"10.1016\/j.ajpath.2017.04.014_bib38","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1084\/jem.192.6.789","article-title":"Regulation of elastinolytic cysteine proteinase activity in normal and cathepsin K\u2013deficient human macrophages","volume":"192","author":"Punturieri","year":"2000","journal-title":"J Exp Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib39","doi-asserted-by":"crossref","first-page":"1595","DOI":"10.1056\/NEJMoa1100391","article-title":"Efficacy and safety of sirolimus in lymphangioleiomyomatosis","volume":"364","author":"McCormack","year":"2011","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib40","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1177\/1536012116645439","article-title":"Assessing metabolic changes in response to mTOR inhibition in a mantle cell lymphoma xenograft model using AcidoCEST MRI","volume":"15","author":"Akhenblit","year":"2016","journal-title":"Mol Imaging"},{"key":"10.1016\/j.ajpath.2017.04.014_bib41","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1007\/s11010-014-2184-2","article-title":"Bicarbonate transport inhibitor SITS modulates pH homeostasis triggering apoptosis of Dalton's lymphoma: implication of novel molecular mechanisms","volume":"397","author":"Kant","year":"2014","journal-title":"Mol Cell Biochem"},{"key":"10.1016\/j.ajpath.2017.04.014_bib42","doi-asserted-by":"crossref","first-page":"1896","DOI":"10.1021\/jm101541x","article-title":"Ureido-substituted benzenesulfonamides potently inhibit carbonic anhydrase IX and show antimetastatic activity in a model of breast cancer metastasis","volume":"54","author":"Pacchiano","year":"2011","journal-title":"J Med Chem"},{"key":"10.1016\/j.ajpath.2017.04.014_bib43","doi-asserted-by":"crossref","first-page":"8271","DOI":"10.1021\/jm200983e","article-title":"Glycosyl coumarin carbonic anhydrase IX and XII inhibitors strongly attenuate the growth of primary breast tumors","volume":"54","author":"Touisni","year":"2011","journal-title":"J Med Chem"},{"key":"10.1016\/j.ajpath.2017.04.014_bib44","doi-asserted-by":"crossref","first-page":"3364","DOI":"10.1158\/0008-5472.CAN-10-4261","article-title":"Targeting tumor hypoxia: suppression of breast tumor growth and metastasis by novel carbonic anhydrase IX inhibitors","volume":"71","author":"Lou","year":"2011","journal-title":"Cancer Res"},{"key":"10.1016\/j.ajpath.2017.04.014_bib45","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1007\/s11914-011-0085-9","article-title":"Inhibition of cathepsin K for treatment of osteoporosis","volume":"10","author":"Boonen","year":"2012","journal-title":"Curr Osteoporos Rep"},{"key":"10.1016\/j.ajpath.2017.04.014_bib46","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1164\/rccm.201205-0848OE","article-title":"Lymphangioleiomyomatosis","volume":"186","author":"McCormack","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.ajpath.2017.04.014_bib47","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1183\/09031936.00210714","article-title":"Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study","volume":"46","author":"Goldberg","year":"2015","journal-title":"Eur Respir J"},{"key":"10.1016\/j.ajpath.2017.04.014_bib48","doi-asserted-by":"crossref","first-page":"1202","DOI":"10.1136\/thoraxjnl-2015-207171","article-title":"A 4-year prospective evaluation of protocols to improve clinical outcomes for patients with lymphangioleiomyomatosis in a national clinical centre","volume":"70","author":"Bee","year":"2015","journal-title":"Thorax"},{"key":"10.1016\/j.ajpath.2017.04.014_bib49","first-page":"162","article-title":"Transcriptional control of the tumor- and hypoxia-marker carbonic anhydrase 9: a one transcription factor (HIF-1) show?","volume":"1795","author":"Kaluz","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.ajpath.2017.04.014_bib50","doi-asserted-by":"crossref","first-page":"3744","DOI":"10.1158\/0008-5472.CAN-15-1862","article-title":"Disrupting hypoxia-induced bicarbonate transport acidifies tumor cells and suppresses tumor growth","volume":"76","author":"McIntyre","year":"2016","journal-title":"Cancer Res"}],"container-title":["The American Journal of Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002944017303711?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002944017303711?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T19:59:03Z","timestamp":1659124743000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0002944017303711"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,8]]},"references-count":50,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2017,8]]}},"alternative-id":["S0002944017303711"],"URL":"https:\/\/doi.org\/10.1016\/j.ajpath.2017.04.014","relation":{},"ISSN":["0002-9440"],"issn-type":[{"value":"0002-9440","type":"print"}],"subject":[],"published":{"date-parts":[[2017,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cathepsin K in Lymphangioleiomyomatosis","name":"articletitle","label":"Article Title"},{"value":"The American Journal of Pathology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ajpath.2017.04.014","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 American Society for Investigative Pathology. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}