{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T20:10:59Z","timestamp":1770063059114,"version":"3.49.0"},"reference-count":43,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2008,9,4]],"date-time":"2008-09-04T00:00:00Z","timestamp":1220486400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2008,12]]},"abstract":"<jats:p>\n                    The metabolism of hyaluronan (HA) re lies on HA synthases and hyaluronidases, among which hyaluronidase\u20101 (HYAL1) and \u20102 (HYAL2) have been proposed as key actors. Congenital HYAL1 deficiency leads to mucopolysaccharidosis IX (MPS IX), a rare lysosomal storage disorder characterized by joint ab normalities. Knowledge of HYAL2 is limited. This protein displays weak\n                    <jats:italic>in vitro<\/jats:italic>\n                    hyaluronidase activity and acts as a receptor for oncogenic ovine retroviruses. We have generated HYAL2\u2010deficient mice through a condi tional Cre\u2010lox system.\n                    <jats:italic>\n                      Hyal2\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice are viable and fertile. They exhibit localized congenital defects in frontonasal and vertebral bone formation and suffer from mild thrombocytopenia and chronic, possibly intravascular, hemolysis. In addition,\n                    <jats:italic>\n                      Hyal2\n                      <jats:sup>\u2010\/\u2010<\/jats:sup>\n                    <\/jats:italic>\n                    mice display 10\u2010fold increases in plasma levels of HA and 2\u2010fold increases in plasma hyaluronidase activity. Glo bally, there is no HA accumulation in tissues, including bones, but liver sinusoidal cells seem overloaded with undigested HA. Taken together, these elements dem onstrate for the first time that murine HYAL2 has a physiological activity\n                    <jats:italic>in vivo<\/jats:italic>\n                    that is relevant for cranio vertebral bone formation, maintenance of plasma HA concentrations, and erythrocyte and platelet homeosta sis. In addition, the viability of HYAL2\u2010deficient mice raises the possibility that a similar defect, defining a new MPS disorder, exists in humans.\u2014 Jadin, L., Wu, X., Ding, H., Frost, G. I., Onclinx, C., Triggs\u2010Raine, B., Flamion, B. Skeletal and hematological anomalies in HYAL2\u2010deficient mice: a second type of mucopolysaccharidosis IX?.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    22, 4316\u20134326 (2008)\n                  <\/jats:p>","DOI":"10.1096\/fj.08-111997","type":"journal-article","created":{"date-parts":[[2008,9,4]],"date-time":"2008-09-04T21:40:35Z","timestamp":1220564435000},"page":"4316-4326","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":87,"title":["Skeletal and hematological anomalies in HYAL2\u2010deficient mice: a second type of mucopolysaccharidosis IX?"],"prefix":"10.1096","volume":"22","author":[{"given":"Laurence","family":"Jadin","sequence":"first","affiliation":[{"name":"URPhyM, Laboratory of Physiology and Pharmacology University of Namur Namur Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoli","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Medical Genetics The University of Manitoba Winnipeg Manitoba Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hao","family":"Ding","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Medical Genetics The University of Manitoba Winnipeg Manitoba Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory I.","family":"Frost","sequence":"additional","affiliation":[{"name":"Halozyme Therapeutics San Diego California USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9cile","family":"Onclinx","sequence":"additional","affiliation":[{"name":"URPhyM, Laboratory of Physiology and Pharmacology University of Namur Namur Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Triggs\u2010Raine","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Medical Genetics The University of Manitoba Winnipeg Manitoba Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno","family":"Flamion","sequence":"additional","affiliation":[{"name":"URPhyM, Laboratory of Physiology and Pharmacology University of Namur Namur Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2008,9,4]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.6.7.1563592"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0955-0674(00)00135-6"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.R700036200"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbagen.2004.02.013"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI10272"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0945-053X(01)00172-X"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mrd.20360"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1095\/biolreprod56.2.320"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1006\/bbrc.1997.6773"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199610033351405"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.11.6296"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn088"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M608358200"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.071572898"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1128\/JVI.02177-06"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1078\/0171-9335-00392"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcb.2006.05.009"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cellbio.23.090506.123337"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0945-053X(01)00170-6"},{"key":"e_1_2_6_21_1","volume-title":"Manipulating the Mouse Embryo: A Laboratory Manual","author":"Nagy A.","year":"2003"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1006\/abio.1997.2262"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s001250050061"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/cwh011"},{"key":"e_1_2_6_25_1","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1007\/BF02986709","article-title":"Genetical studies on the skeleton of the mouse. 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