{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T03:59:33Z","timestamp":1779335973792,"version":"3.51.4"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2000,3,1]],"date-time":"2000-03-01T00:00:00Z","timestamp":951868800000},"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":[[2000,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Brain tissue from patients with classical late\u2010infantile neuronal ceroid lipofuscinosis (LINCL, an infantile form of Batten disease) is deficient in the lysosomal enzyme tripeptidyl\u2010peptidase I (EC 3.4.14.9). The activities of other lysosomal enzymes are either increased or decreased. Tripeptidyl\u2010peptidase I is a pepstatin\u2010insensitive exo\u2010tripeptidase, with little or no endo\u2010proteolytic activity, that is active on small peptides but not on large proteins. Using haemoglobin and casein as substrates for proteolytic activity, we were unable to demonstrate any significant defect in pepstatin\u2010sensitive or pepstatin\u2010insensitive proteinase activity in brain tissue or cultured skin fibroblasts of LINCL patients. These observations suggest that the lysosomal storage of undegraded, small peptides in LINCL results from the absence of peptidase rather than proteinase activity.<\/jats:p>","DOI":"10.1023\/a:1005665732189","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T23:07:17Z","timestamp":1040512037000},"page":"145-154","source":"Crossref","is-referenced-by-count":17,"title":["Tripeptidyl\u2010peptidase I deficiency in classical late\u2010infantile neuronal ceroid lipofuscinosis brain tissue. Evidence for defective peptidase rather than proteinase activity"],"prefix":"10.1002","volume":"23","author":[{"given":"M. J.","family":"Warburton","sequence":"first","affiliation":[{"name":"Department of Histopathology St George's Hospital Medical School  London SW17 0RE UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Bernardini","sequence":"additional","affiliation":[{"name":"Department of Histopathology St George's Hospital Medical School  London SW17 0RE UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2000,3]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"671","volume-title":"Handbook of Clinical Neurology, vol. 22: Neurodystrophies and Neurolipidoses","author":"Boustany R\u2010M","year":"1996"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1996.67041677.x"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.283.5404.978"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.1998.tb00142.x"},{"key":"e_1_2_1_6_1","first-page":"218","volume-title":"Lysosomes and Storage Diseases","author":"Hers HG","year":"1973"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.jbchem.a123749"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1998.5328"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1006\/nbdi.1998.0209"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1006\/abbi.1993.1523"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/380403a0"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.277.5333.1802"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1999.0730700.x"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.178.4063.871"},{"key":"e_1_2_1_15_1","volume-title":"The purification and characterisation of tripeptidyl peptidase\u2010I from spleen.","author":"Vines DJ","year":"1997"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-4838(98)00012-0"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(98)01683-4"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(82)90482-1"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005665732189","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005665732189","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T21:26:37Z","timestamp":1694813197000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005665732189"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2000,3]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2000,3]]}},"alternative-id":["10.1023\/A:1005665732189"],"URL":"https:\/\/doi.org\/10.1023\/a:1005665732189","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2000,3]]}}}