{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T09:36:05Z","timestamp":1779183365227,"version":"3.51.4"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2005,12,1]],"date-time":"2005-12-01T00:00:00Z","timestamp":1133395200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2005,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Bone marrow transplantation is the therapy of choice in patients affected by MPS I (Hurler syndrome), but a high incidence of rejection limits the success of this treatment. The deficiency of \u03b1\u2010<jats:sc>L<\/jats:sc>\u2010iduronidase (EC 1.2.3.76), one of the enzymes responsible for the degradation of glycosaminoglycans, results in accumulation of heparan and dermatan sulphate in these patients. Heparan sulphate and dermatan sulphate are known to be important components of the bone marrow microenvironment and critical for haematopoietic cell development. In this study we compared the ability of marrow stromal cells from MPS I patients and healthy donors to support normal haematopoiesis in Dexter\u2010type long term culture. We found an inverse stroma\/supernatant ratio in the number of clonogenic progenitors, particularly the colony\u2010forming unit granulocyte\u2013machrophage in MPS I cultures when compared to normal controls. No alteration in the adhesion of haematopoietic cells to the stroma of MPS I patients was found, suggesting that the altered distribution in the number of clonogenic progenitors is probably the result of an accelerated process of differentiation and maturation. The use of \u03b1\u2010<jats:sc>L<\/jats:sc>\u2010iduronidase gene\u2010corrected marrow stromal cells re\u2010established normal haematopoiesis in culture, suggesting that correction of the bone marrow microenvironment with competent enzyme prior to transplantation might help establishment of donor haematopoiesis.<\/jats:p>","DOI":"10.1007\/s10545-005-0136-4","type":"journal-article","created":{"date-parts":[[2006,1,25]],"date-time":"2006-01-25T09:35:29Z","timestamp":1138181729000},"page":"1045-1053","source":"Crossref","is-referenced-by-count":20,"title":["Marrow stromal cells from patients affected by MPS I differentially support haematopoietic progenitor cell development"],"prefix":"10.1002","volume":"28","author":[{"given":"M. A.","family":"Baxter","sequence":"first","affiliation":[{"name":"Stem Cell Research Group Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R. 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J.","family":"Fairbairn","sequence":"additional","affiliation":[{"name":"Cancer Research UK Gene Therapy Group Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Bellantuono","sequence":"additional","affiliation":[{"name":"Stem Cell Research Group Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,12]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.5.1857"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI10413"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4644(199702)64:2<278::AID-JCB11>3.0.CO;2-F"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature02040"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-0528.2002.t01-1-02011.x"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V98.8.2396"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0950-3536(11)80010-7"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.5.2025"},{"key":"e_1_2_1_10_1","first-page":"1347","article-title":"Phenotype and progeny of primitive adherent human hematopoietic progenitors","volume":"10","author":"Gordon MY","year":"1996","journal-title":"Leukemia"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V95.1.147"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64961-9"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(81)91046-1"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-08-2597"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118063"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.165.3.1599"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI0215272"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002239900656"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-02-0657"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.11.4894.bloodjournal87114894"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V91.7.2601"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/jor.1100090504"},{"key":"e_1_2_1_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/0014-5793(96)00240-2"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1096-7192(03)00018-0"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V80.4.912.912"},{"key":"e_1_2_1_26_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.165.4.1863"},{"key":"e_1_2_1_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/0145-2126(80)90027-2"},{"key":"e_1_2_1_28_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.76.2.92"},{"key":"e_1_2_1_29_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V84.6.1802.1802"},{"key":"e_1_2_1_30_1","first-page":"7","article-title":"Bone marrow transplantation for Hurler syndrome: assessment of metabolic correction","volume":"22","author":"Whitley CB","year":"1986","journal-title":"Birth Defects Orig Artic Ser"},{"key":"e_1_2_1_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature02041"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-005-0136-4","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-005-0136-4","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T02:16:37Z","timestamp":1694744197000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-005-0136-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,12]]},"references-count":30,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2005,12]]}},"alternative-id":["10.1007\/s10545-005-0136-4"],"URL":"https:\/\/doi.org\/10.1007\/s10545-005-0136-4","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,12]]}}}