{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T11:03:01Z","timestamp":1789988581015,"version":"4.0.1"},"reference-count":25,"publisher":"American Chemical Society (ACS)","issue":"15","license":[{"start":{"date-parts":[[2015,7,14]],"date-time":"2015-07-14T00:00:00Z","timestamp":1436832000000},"content-version":"vor","delay-in-days":365,"URL":"https:\/\/pubs.acs.org\/page\/policy\/authorchoice_termsofuse.html"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 DK067859"],"award-info":[{"award-number":["R01 DK067859"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,8,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>We report new substrates for quantitative enzyme activity measurements of human palmitoyl protein thioesterase (PPT1) and tripeptidyl peptidase (TPP1) in dried blood spots from newborns using tandem mass spectrometry. Deficiencies in these enzyme activities due to inborn errors of metabolism cause neuronal ceroid lipofuscinoses. The assays use synthetic compounds that were designed to mimic the natural substrates. Incubation produces nanomole quantities of enzymatic products per a blood spot that are quantified by tandem mass spectrometry using synthetic internal standards and selected reaction monitoring. The assays utilize a minimum steps for sample workup and can be run in a duplex format for the detection of neuronal ceroid lipofuscinoses or potentially multiplexed with other mass spectrometry-based assays for newborn screening of lysosomal storage disorders.<\/jats:p>","DOI":"10.1021\/ac501994b","type":"journal-article","created":{"date-parts":[[2014,7,14]],"date-time":"2014-07-14T18:59:03Z","timestamp":1405364343000},"page":"7962-7968","source":"Crossref","is-referenced-by-count":18,"title":["Tandem Mass Spectrometry Assays of Palmitoyl Protein\nThioesterase 1 and Tripeptidyl Peptidase Activity in Dried Blood Spots\nfor the Detection of Neuronal Ceroid Lipofuscinoses in Newborns"],"prefix":"10.1021","volume":"86","author":[{"given":"Mariana","family":"Barcenas","sequence":"first","affiliation":[{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang","family":"Xue","sequence":"additional","affiliation":[{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatyana","family":"Marushchak-Vlaskin","sequence":"additional","affiliation":[{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. Ronald","family":"Scott","sequence":"additional","affiliation":[{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael H.","family":"Gelb","sequence":"additional","affiliation":[{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]},{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frantis\u030cek","family":"Turec\u030cek","sequence":"additional","affiliation":[{"name":"University of Washington , , , ,","place":["Seattle, Washington, United States, 98115-1700"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"316","published-online":{"date-parts":[[2014,7,14]]},"reference":[{"key":"2026092106130938800_cit1","first-page":"3877","article-title":"The Neuronal Ceroid\nLipofuscinoses","volume-title":"The Metabolic and Molecular\nBasis of Inherited Disease","author":"Hofmann","year":"2001","edition":"8"},{"key":"2026092106130938800_cit2","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1111\/j.1469-8749.1974.tb04183.x","volume":"16","author":"Santavuori","year":"1974","journal-title":"Dev. 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