Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases

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...

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Main Authors: Agnieszka Ługowska, Galina Baydakova, Alex Ilyushkina, Ekaterina Zakharova, Hanna Mierzewska, Krystyna Szymańska, Jolanta Wierzba, Jolanta Kubalska, Ałła Graban, Tomasz Kmieć, Barbara Perkowska-Sumiła, Anna Tylki-Szymańska, Małgorzata Bednarska-Makaruk
Format: Article
Language:English
Published: MDPI AG 2021-02-01
Series:Diagnostics
Subjects:
Online Access:https://www.mdpi.com/2075-4418/11/2/320
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author Agnieszka Ługowska
Galina Baydakova
Alex Ilyushkina
Ekaterina Zakharova
Hanna Mierzewska
Krystyna Szymańska
Jolanta Wierzba
Jolanta Kubalska
Ałła Graban
Tomasz Kmieć
Barbara Perkowska-Sumiła
Anna Tylki-Szymańska
Małgorzata Bednarska-Makaruk
author_facet Agnieszka Ługowska
Galina Baydakova
Alex Ilyushkina
Ekaterina Zakharova
Hanna Mierzewska
Krystyna Szymańska
Jolanta Wierzba
Jolanta Kubalska
Ałła Graban
Tomasz Kmieć
Barbara Perkowska-Sumiła
Anna Tylki-Szymańska
Małgorzata Bednarska-Makaruk
author_sort Agnieszka Ługowska
collection DOAJ
description 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 (<i>p</i> < 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–Pick 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.
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spelling doaj.art-e50ce5218c0644baaf48e583eb8bd2692023-12-11T17:18:17ZengMDPI AGDiagnostics2075-44182021-02-0111232010.3390/diagnostics11020320Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal DiseasesAgnieszka Ługowska0Galina Baydakova1Alex Ilyushkina2Ekaterina Zakharova3Hanna Mierzewska4Krystyna Szymańska5Jolanta Wierzba6Jolanta Kubalska7Ałła Graban8Tomasz Kmieć9Barbara Perkowska-Sumiła10Anna Tylki-Szymańska11Małgorzata Bednarska-Makaruk12Department of Genetics, Institute of Psychiatry and Neurology, 02-957 Warsaw, PolandResearch Centre for Medical Genetics, Federal State Budgetary Institution, 115478 Moscow, RussiaResearch Centre for Medical Genetics, Federal State Budgetary Institution, 115478 Moscow, RussiaResearch Centre for Medical Genetics, Federal State Budgetary Institution, 115478 Moscow, RussiaDepartment of Child and Adolescent Neurology, Institute of Mother and Child, 01-211 Warsaw, PolandMossakowski Medical Research Center, Department of Experimental and Clinical Neuropathology, Polish Academy of Sciences, 02-106 Warsaw, PolandDepartment of Internal and Pediatric Nursing, Institute of Nursing and Midwifery, Medical University of Gdańsk, 80-210 Gdańsk, PolandDepartment of Genetics, Institute of Psychiatry and Neurology, 02-957 Warsaw, Poland1st Department of Neurology, Institute of Psychiatry and Neurology, 02-957 Warsaw, PolandDepartment of Neurology and Epileptology, The Children’s Memorial Health Institute, 04-730 Warsaw, PolandDepartment of Pediatrics, Nutrition and Metabolic Diseases, The Children’s Memorial Health Institute, 04-730 Warsaw, PolandDepartment of Pediatrics, Nutrition and Metabolic Diseases, The Children’s Memorial Health Institute, 04-730 Warsaw, PolandDepartment of Genetics, Institute of Psychiatry and Neurology, 02-957 Warsaw, PolandIncreased 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 (<i>p</i> < 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–Pick 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.https://www.mdpi.com/2075-4418/11/2/320DPP-IVlysosomal diseasesmucolipidosis II/IIImucopolysaccharidosesalpha-mannosidosisdiagnosis
spellingShingle Agnieszka Ługowska
Galina Baydakova
Alex Ilyushkina
Ekaterina Zakharova
Hanna Mierzewska
Krystyna Szymańska
Jolanta Wierzba
Jolanta Kubalska
Ałła Graban
Tomasz Kmieć
Barbara Perkowska-Sumiła
Anna Tylki-Szymańska
Małgorzata Bednarska-Makaruk
Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases
Diagnostics
DPP-IV
lysosomal diseases
mucolipidosis II/III
mucopolysaccharidoses
alpha-mannosidosis
diagnosis
title Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases
title_full Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases
title_fullStr Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases
title_full_unstemmed Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases
title_short Elevated Dipeptidyl Peptidase IV (DPP-IV) Activity in Plasma from Patients with Various Lysosomal Diseases
title_sort elevated dipeptidyl peptidase iv dpp iv activity in plasma from patients with various lysosomal diseases
topic DPP-IV
lysosomal diseases
mucolipidosis II/III
mucopolysaccharidoses
alpha-mannosidosis
diagnosis
url https://www.mdpi.com/2075-4418/11/2/320
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