{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T01:49:11Z","timestamp":1760233751506,"version":"build-2065373602"},"reference-count":24,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,2,16]],"date-time":"2021-02-16T00:00:00Z","timestamp":1613433600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Increased activity of dipeptidyl peptidase IV (DPP-IV) was reported earlier in patients with different types of mucopolysaccharidoses. DPP-IV (also known as CD26 lymphocyte T surface antigen) is a transmembrane protein showing protease activity. This enzyme displays various functions in the organism and plays an important role in multiple processes like glucose metabolism, nociception, cell-adhesion, psychoneuroendocrine regulation, immune response and cardiovascular adaptation. In order to evaluate DPP-IV in lysosomal storage diseases (LSD), we examined its activity in plasma samples from 307 patients affected with 24 different LSDs and in 75 control persons. Our results revealed elevated DPP-IV activity especially in individuals affected with mucolipidosis II\/III, alpha-mannosidosis, and mucopolysaccharidoses types III, II, and I (p &lt; 0.05). In other LSDs the DPP-IV activity was still significantly increased, but to a lesser extent. In patients with Gaucher disease, ceroid lipofuscinosis type 1 (CLN1), Niemann\u2013Pick disease type C and A, Krabbe and Pompe diseases, gangliosidosis GM2 and metachromatic leukodystrophy discreet or no changes in DPP-IV activity were observed. DPP-IV may serve as a first-tier diagnostic procedure or additional biochemical analysis in recognizing patients with some LSDs. DPP-IV may become an object of basic research for a better understanding of LSDs.<\/jats:p>","DOI":"10.3390\/diagnostics11020320","type":"journal-article","created":{"date-parts":[[2021,2,16]],"date-time":"2021-02-16T22:13:38Z","timestamp":1613513618000},"page":"320","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2848-6407","authenticated-orcid":false,"given":"Agnieszka","family":"\u0141ugowska","sequence":"first","affiliation":[{"name":"Department of Genetics, Institute of Psychiatry and Neurology, 02-957 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Galina","family":"Baydakova","sequence":"additional","affiliation":[{"name":"Research Centre for Medical Genetics, Federal State Budgetary Institution, 115478 Moscow, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alex","family":"Ilyushkina","sequence":"additional","affiliation":[{"name":"Research Centre for Medical Genetics, Federal State Budgetary Institution, 115478 Moscow, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ekaterina","family":"Zakharova","sequence":"additional","affiliation":[{"name":"Research Centre for Medical Genetics, Federal State Budgetary Institution, 115478 Moscow, Russia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hanna","family":"Mierzewska","sequence":"additional","affiliation":[{"name":"Department of Child and Adolescent Neurology, Institute of Mother and Child, 01-211 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4306-2570","authenticated-orcid":false,"given":"Krystyna","family":"Szyma\u0144ska","sequence":"additional","affiliation":[{"name":"Mossakowski Medical Research Center, Department of Experimental and Clinical Neuropathology, Polish Academy of Sciences, 02-106 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jolanta","family":"Wierzba","sequence":"additional","affiliation":[{"name":"Department of Internal and Pediatric Nursing, Institute of Nursing and Midwifery, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jolanta","family":"Kubalska","sequence":"additional","affiliation":[{"name":"Department of Genetics, Institute of Psychiatry and Neurology, 02-957 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A\u0142\u0142a","family":"Graban","sequence":"additional","affiliation":[{"name":"1st Department of Neurology, Institute of Psychiatry and Neurology, 02-957 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomasz","family":"Kmie\u0107","sequence":"additional","affiliation":[{"name":"Department of Neurology and Epileptology, The Children\u2019s Memorial Health Institute, 04-730 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Perkowska-Sumi\u0142a","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nutrition and Metabolic Diseases, The Children\u2019s Memorial Health Institute, 04-730 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Tylki-Szyma\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nutrition and Metabolic Diseases, The Children\u2019s Memorial Health Institute, 04-730 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ma\u0142gorzata","family":"Bednarska-Makaruk","sequence":"additional","affiliation":[{"name":"Department of Genetics, Institute of Psychiatry and Neurology, 02-957 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1001\/jama.281.3.249","article-title":"Prevalence of Lysosomal Storage disorders","volume":"281","author":"Meikle","year":"1999","journal-title":"JAMA"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1038\/sj.ejhg.5201044","article-title":"Prevalence of lysosomal storage diseases in Portugal","volume":"12","author":"Pinto","year":"2004","journal-title":"Eur. 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