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Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report

Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder that occurs due to a deficiency of a β hexosaminidase A (HexA) enzyme, resulting in the accumulation of GM(2) gangliosides. In this work, we analyzed the effect of umbilical cord blood cell transplantation (UCBCT) and curcumin admin...

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Autores principales: Shaimardanova, Alisa A., Chulpanova, Daria S., Solovyeva, Valeriya V., Garanina, Ekaterina E., Salafutdinov, Ilnur I., Laikov, Alexander Vladimirovich, Kursenko, Vadim V., Chakrabarti, Lisa, Zakharova, Ekaterina Yu., Bukina, Tatiana M., Baydakova, Galina V., Rizvanov, Albert Anatolyevich
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8539434/
https://www.ncbi.nlm.nih.gov/pubmed/34685379
http://dx.doi.org/10.3390/life11101007
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author Shaimardanova, Alisa A.
Chulpanova, Daria S.
Solovyeva, Valeriya V.
Garanina, Ekaterina E.
Salafutdinov, Ilnur I.
Laikov, Alexander Vladimirovich
Kursenko, Vadim V.
Chakrabarti, Lisa
Zakharova, Ekaterina Yu.
Bukina, Tatiana M.
Baydakova, Galina V.
Rizvanov, Albert Anatolyevich
author_facet Shaimardanova, Alisa A.
Chulpanova, Daria S.
Solovyeva, Valeriya V.
Garanina, Ekaterina E.
Salafutdinov, Ilnur I.
Laikov, Alexander Vladimirovich
Kursenko, Vadim V.
Chakrabarti, Lisa
Zakharova, Ekaterina Yu.
Bukina, Tatiana M.
Baydakova, Galina V.
Rizvanov, Albert Anatolyevich
author_sort Shaimardanova, Alisa A.
collection PubMed
description Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder that occurs due to a deficiency of a β hexosaminidase A (HexA) enzyme, resulting in the accumulation of GM(2) gangliosides. In this work, we analyzed the effect of umbilical cord blood cell transplantation (UCBCT) and curcumin administration on the course of the disease in a patient with adult TSD. The patient’s serum cytokine profile was determined using multiplex analysis. The level of GM(2) gangliosides in plasma was determined using mass spectrometry. The enzymatic activity of HexA in the plasma of the patient was assessed using a fluorescent substrate assay. The HexA α-subunit (HexA) concentration was determined using ELISA. It was shown that both UCBCT and curcumin administration led to a change in the patient’s cytokine profile. The UCBCT resulted in an increase in the concentration of HexA in the patient’s serum and in an improvement in the patient’s neurological status. However, neither UCBCT nor curcumin were able to alter HexA activity and the level of GM(2) in patient’s plasma. The data obtained indicate that UCBCT and curcumin administration can alter the immunity of a patient with TSD, reduce the level of inflammatory cytokines and thereby improve the patient’s condition.
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spelling pubmed-85394342021-10-24 Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report Shaimardanova, Alisa A. Chulpanova, Daria S. Solovyeva, Valeriya V. Garanina, Ekaterina E. Salafutdinov, Ilnur I. Laikov, Alexander Vladimirovich Kursenko, Vadim V. Chakrabarti, Lisa Zakharova, Ekaterina Yu. Bukina, Tatiana M. Baydakova, Galina V. Rizvanov, Albert Anatolyevich Life (Basel) Case Report Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder that occurs due to a deficiency of a β hexosaminidase A (HexA) enzyme, resulting in the accumulation of GM(2) gangliosides. In this work, we analyzed the effect of umbilical cord blood cell transplantation (UCBCT) and curcumin administration on the course of the disease in a patient with adult TSD. The patient’s serum cytokine profile was determined using multiplex analysis. The level of GM(2) gangliosides in plasma was determined using mass spectrometry. The enzymatic activity of HexA in the plasma of the patient was assessed using a fluorescent substrate assay. The HexA α-subunit (HexA) concentration was determined using ELISA. It was shown that both UCBCT and curcumin administration led to a change in the patient’s cytokine profile. The UCBCT resulted in an increase in the concentration of HexA in the patient’s serum and in an improvement in the patient’s neurological status. However, neither UCBCT nor curcumin were able to alter HexA activity and the level of GM(2) in patient’s plasma. The data obtained indicate that UCBCT and curcumin administration can alter the immunity of a patient with TSD, reduce the level of inflammatory cytokines and thereby improve the patient’s condition. MDPI 2021-09-24 /pmc/articles/PMC8539434/ /pubmed/34685379 http://dx.doi.org/10.3390/life11101007 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Shaimardanova, Alisa A.
Chulpanova, Daria S.
Solovyeva, Valeriya V.
Garanina, Ekaterina E.
Salafutdinov, Ilnur I.
Laikov, Alexander Vladimirovich
Kursenko, Vadim V.
Chakrabarti, Lisa
Zakharova, Ekaterina Yu.
Bukina, Tatiana M.
Baydakova, Galina V.
Rizvanov, Albert Anatolyevich
Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report
title Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report
title_full Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report
title_fullStr Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report
title_full_unstemmed Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report
title_short Serum Cytokine Profile, Beta-Hexosaminidase A Enzymatic Activity and GM(2) Ganglioside Levels in the Plasma of a Tay-Sachs Disease Patient after Cord Blood Cell Transplantation and Curcumin Administration: A Case Report
title_sort serum cytokine profile, beta-hexosaminidase a enzymatic activity and gm(2) ganglioside levels in the plasma of a tay-sachs disease patient after cord blood cell transplantation and curcumin administration: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8539434/
https://www.ncbi.nlm.nih.gov/pubmed/34685379
http://dx.doi.org/10.3390/life11101007
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