{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T22:13:25Z","timestamp":1768515205435,"version":"3.49.0"},"reference-count":42,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2024,6,6]],"date-time":"2024-06-06T00:00:00Z","timestamp":1717632000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006769","name":"Russian Science Foundation","doi-asserted-by":"publisher","award":["22-79-10187"],"award-info":[{"award-number":["22-79-10187"]}],"id":[{"id":"10.13039\/501100006769","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Sensors"],"abstract":"<jats:p>Fabry disease is a lysosomal storage disorder caused by a significant decrease in the activity or absence of the enzyme \u03b1-galactosidase A. The diagnostics of Fabry disease during newborn screening are reasonable, due to the availability of enzyme replacement therapy. This paper presents an electrochemical method using complementary metal-oxide semiconductor (CMOS)-compatible ion-sensitive field effect transistors (ISFETs) with hafnium oxide-sensitive surfaces for the detection of \u03b1-galactosidase A activity in dried blood spot extracts. The capability of ISFETs to detect the reaction catalyzed by \u03b1-galactosidase A was demonstrated. The buffer composition was optimized to provide suitable conditions for both enzyme and ISFET performance. The use of ISFET structures as sensor elements allowed for the label-free detection of enzymatic reactions with melibiose, a natural substrate of \u03b1-galactosidase A, instead of a synthetic fluorogenic one. ISFET chips were packaged with printed circuit boards and microfluidic reaction chambers to enable long-term signal measurement using a custom device. The packaged sensors were demonstrated to discriminate between normal and inhibited GLA activity in dried blood spots extracts. The described method offers a promising solution for increasing the widespread distribution of newborn screening of Fabry disease.<\/jats:p>","DOI":"10.3390\/s24113681","type":"journal-article","created":{"date-parts":[[2024,6,6]],"date-time":"2024-06-06T06:40:26Z","timestamp":1717656026000},"page":"3681","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Detection of \u03b1-Galactosidase A Reaction in Samples Extracted from Dried Blood Spots Using Ion-Sensitive Field Effect Transistors"],"prefix":"10.3390","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1333-5294","authenticated-orcid":false,"given":"Alexander","family":"Kuznetsov","sequence":"first","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-6114-010X","authenticated-orcid":false,"given":"Andrey","family":"Sheshil","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eugene","family":"Smolin","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9564-5009","authenticated-orcid":false,"given":"Vitaliy","family":"Grudtsov","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dmitriy","family":"Ryazantsev","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9583-1998","authenticated-orcid":false,"given":"Mark","family":"Shustinskiy","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatiana","family":"Tikhonova","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Irakli","family":"Kitiashvili","sequence":"additional","affiliation":[{"name":"Municipal Clinical Hospital No. 15 named after O.M. Filatov, 23 Veshnyakovskaya St., Moscow 111539, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valerii","family":"Vechorko","sequence":"additional","affiliation":[{"name":"Municipal Clinical Hospital No. 15 named after O.M. Filatov, 23 Veshnyakovskaya St., Moscow 111539, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6148-0971","authenticated-orcid":false,"given":"Natalia","family":"Komarova","sequence":"additional","affiliation":[{"name":"Institute of Nanotechnology of Microelectronics of the Russian Academy of Sciences, 32A Leninsky Prospekt, Moscow 119334, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1002\/bdr2.1653","article-title":"Mandatory Newborn Screening in the United States: History, Current Status, and Existential Challenges","volume":"112","author":"McCandless","year":"2020","journal-title":"Birth Defects Res."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Gragnaniello, V., Burlina, A.B.A.P., Polo, G., Giuliani, A., Salviati, L., Duro, G., Cazzorla, C., Rubert, L., Maines, E., and Germain, D.P. 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