Decreased Serum Levels of Sphingomyelins and Ceramides in Sickle Cell Disease Patients

Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle‐cell disease (SCD). This study was aimed at identifying the levels of C16–C24 CerPCho and C16–C24 CER in serum obtained from SCD patients and controls. Circulating levels of neutr...

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Vydané v:	Lipids Ročník 53; číslo 3; s. 313 - 322
Hlavní autori:	Aslan, Mutay, Kıraç, Ebru, Kaya, Sabriye, Özcan, Filiz, Salim, Ozan, Küpesiz, Osman Alphan
Médium:	Journal Article
Jazyk:	English
Vydavateľské údaje:	Hoboken, USA John Wiley & Sons, Inc 01.03.2018
Predmet:	Adolescent Anemia, Sickle Cell - blood blood serum Case-Control Studies Ceramide ceramides Ceramides - blood Ceramides - classification Child cholesterol Cholesterol, HDL - blood Cholesterol, LDL - blood Cholesterol, VLDL - blood Chromatography, High Pressure Liquid enzyme-linked immunosorbent assay Female hemoglobin homozygosity Humans Lysophospholipids - blood Male Neutral sphingomyelinase pathophysiology sickle cell anemia Sickle cell disease Sphingomyelin Sphingomyelin Phosphodiesterase - blood sphingomyelins Sphingomyelins - blood Sphingomyelins - classification Sphingosine - analogs & derivatives Sphingosine - blood Tandem Mass Spectrometry Triglycerides - blood ultrafast liquid chromatography Ceramide Sphingomyelin Sickle cell disease Neutral sphingomyelinase
ISSN:	0024-4201, 1558-9307, 1558-9307
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Abstract	Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle‐cell disease (SCD). This study was aimed at identifying the levels of C16–C24 CerPCho and C16–C24 CER in serum obtained from SCD patients and controls. Circulating levels of neutral sphingomyelinase (N‐SMase) activity, ceramide‐1‐phosphate (C1P), and sphingosine‐1‐phosphate (S1P) were also determined. Blood was collected from 35 hemoglobin (Hb)A volunteers and 45 homozygous HbSS patients. Serum levels of C16–C24 CerPCho and C16–C24 CER were determined by an optimized multiple reaction monitoring (MRM) method using ultrafast liquid chromatography (UFLC) coupled with tandem mass spectrometry (MS/MS). Serum activity of N‐SMase was assayed by standard kit methods, and C1P and S1P levels were determined by enzyme‐linked immunosorbent assay. A significant decrease was observed in the serum levels of C18–C24 CerPCho in patients with SCD compared to controls. No significant difference was found in C16 CerPCho levels between the two groups. Very‐long‐chain C22–C24 CER were significantly decreased in SCD, while long‐chain C16–C20 CER levels showed no significant difference between SCD patients and controls. Significant positive correlation was found between the serum total cholesterol levels and C18–C24 CerPCho and C22–C24 CER in SCD patients. Patients with SCD had significantly elevated serum activity of N‐SMase as well as increased circulating levels of C1P and S1P compared to controls. The decrease in serum levels of C18–C24 CerPCho in patients with SCD was accompanied by decreased levels of C22–C24 CER. Future studies are needed to understand the role of decreased CerPCho and CER in the pathophysiology of SCD.
AbstractList	Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle-cell disease (SCD). This study was aimed at identifying the levels of C16-C24 CerPCho and C16-C24 CER in serum obtained from SCD patients and controls. Circulating levels of neutral sphingomyelinase (N-SMase) activity, ceramide-1-phosphate (C1P), and sphingosine-1-phosphate (S1P) were also determined. Blood was collected from 35 hemoglobin (Hb)A volunteers and 45 homozygous HbSS patients. Serum levels of C16-C24 CerPCho and C16-C24 CER were determined by an optimized multiple reaction monitoring (MRM) method using ultrafast liquid chromatography (UFLC) coupled with tandem mass spectrometry (MS/MS). Serum activity of N-SMase was assayed by standard kit methods, and C1P and S1P levels were determined by enzyme-linked immunosorbent assay. A significant decrease was observed in the serum levels of C18-C24 CerPCho in patients with SCD compared to controls. No significant difference was found in C16 CerPCho levels between the two groups. Very-long-chain C22-C24 CER were significantly decreased in SCD, while long-chain C16-C20 CER levels showed no significant difference between SCD patients and controls. Significant positive correlation was found between the serum total cholesterol levels and C18-C24 CerPCho and C22-C24 CER in SCD patients. Patients with SCD had significantly elevated serum activity of N-SMase as well as increased circulating levels of C1P and S1P compared to controls. The decrease in serum levels of C18-C24 CerPCho in patients with SCD was accompanied by decreased levels of C22-C24 CER. Future studies are needed to understand the role of decreased CerPCho and CER in the pathophysiology of SCD. Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle-cell disease (SCD). This study was aimed at identifying the levels of C16-C24 CerPCho and C16-C24 CER in serum obtained from SCD patients and controls. Circulating levels of neutral sphingomyelinase (N-SMase) activity, ceramide-1-phosphate (C1P), and sphingosine-1-phosphate (S1P) were also determined. Blood was collected from 35 hemoglobin (Hb)A volunteers and 45 homozygous HbSS patients. Serum levels of C16-C24 CerPCho and C16-C24 CER were determined by an optimized multiple reaction monitoring (MRM) method using ultrafast liquid chromatography (UFLC) coupled with tandem mass spectrometry (MS/MS). Serum activity of N-SMase was assayed by standard kit methods, and C1P and S1P levels were determined by enzyme-linked immunosorbent assay. A significant decrease was observed in the serum levels of C18-C24 CerPCho in patients with SCD compared to controls. No significant difference was found in C16 CerPCho levels between the two groups. Very-long-chain C22-C24 CER were significantly decreased in SCD, while long-chain C16-C20 CER levels showed no significant difference between SCD patients and controls. Significant positive correlation was found between the serum total cholesterol levels and C18-C24 CerPCho and C22-C24 CER in SCD patients. Patients with SCD had significantly elevated serum activity of N-SMase as well as increased circulating levels of C1P and S1P compared to controls. The decrease in serum levels of C18-C24 CerPCho in patients with SCD was accompanied by decreased levels of C22-C24 CER. Future studies are needed to understand the role of decreased CerPCho and CER in the pathophysiology of SCD.Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle-cell disease (SCD). This study was aimed at identifying the levels of C16-C24 CerPCho and C16-C24 CER in serum obtained from SCD patients and controls. Circulating levels of neutral sphingomyelinase (N-SMase) activity, ceramide-1-phosphate (C1P), and sphingosine-1-phosphate (S1P) were also determined. Blood was collected from 35 hemoglobin (Hb)A volunteers and 45 homozygous HbSS patients. Serum levels of C16-C24 CerPCho and C16-C24 CER were determined by an optimized multiple reaction monitoring (MRM) method using ultrafast liquid chromatography (UFLC) coupled with tandem mass spectrometry (MS/MS). Serum activity of N-SMase was assayed by standard kit methods, and C1P and S1P levels were determined by enzyme-linked immunosorbent assay. A significant decrease was observed in the serum levels of C18-C24 CerPCho in patients with SCD compared to controls. No significant difference was found in C16 CerPCho levels between the two groups. Very-long-chain C22-C24 CER were significantly decreased in SCD, while long-chain C16-C20 CER levels showed no significant difference between SCD patients and controls. Significant positive correlation was found between the serum total cholesterol levels and C18-C24 CerPCho and C22-C24 CER in SCD patients. Patients with SCD had significantly elevated serum activity of N-SMase as well as increased circulating levels of C1P and S1P compared to controls. The decrease in serum levels of C18-C24 CerPCho in patients with SCD was accompanied by decreased levels of C22-C24 CER. Future studies are needed to understand the role of decreased CerPCho and CER in the pathophysiology of SCD.
Author	Kaya, Sabriye Kıraç, Ebru Aslan, Mutay Küpesiz, Osman Alphan Özcan, Filiz Salim, Ozan
Author_xml	– sequence: 1 givenname: Mutay orcidid: 0000-0002-0660-971X surname: Aslan fullname: Aslan, Mutay email: mutayaslan@akdeniz.edu.tr organization: Akdeniz University Medical School – sequence: 2 givenname: Ebru surname: Kıraç fullname: Kıraç, Ebru organization: Akdeniz University Medical School – sequence: 3 givenname: Sabriye surname: Kaya fullname: Kaya, Sabriye organization: Akdeniz University Medical School – sequence: 4 givenname: Filiz surname: Özcan fullname: Özcan, Filiz organization: Akdeniz University Medical School – sequence: 5 givenname: Ozan surname: Salim fullname: Salim, Ozan organization: Akdeniz University, Faculty of Medicine – sequence: 6 givenname: Osman Alphan surname: Küpesiz fullname: Küpesiz, Osman Alphan organization: Akdeniz University Medical School
BackLink	https://www.ncbi.nlm.nih.gov/pubmed/29663386$$D View this record in MEDLINE/PubMed
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Keywords	Ceramide Sphingomyelin Sickle cell disease Neutral sphingomyelinase
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Snippet	Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle‐cell disease (SCD). This study was... Limited data are available on the serum levels of different sphingomyelin (CerPCho) and ceramide (CER) species in sickle-cell disease (SCD). This study was...
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SubjectTerms	Adolescent Anemia, Sickle Cell - blood blood serum Case-Control Studies Ceramide ceramides Ceramides - blood Ceramides - classification Child cholesterol Cholesterol, HDL - blood Cholesterol, LDL - blood Cholesterol, VLDL - blood Chromatography, High Pressure Liquid enzyme-linked immunosorbent assay Female hemoglobin homozygosity Humans Lysophospholipids - blood Male Neutral sphingomyelinase pathophysiology sickle cell anemia Sickle cell disease Sphingomyelin Sphingomyelin Phosphodiesterase - blood sphingomyelins Sphingomyelins - blood Sphingomyelins - classification Sphingosine - analogs & derivatives Sphingosine - blood Tandem Mass Spectrometry Triglycerides - blood ultrafast liquid chromatography
Title	Decreased Serum Levels of Sphingomyelins and Ceramides in Sickle Cell Disease Patients
URI	https://onlinelibrary.wiley.com/doi/abs/10.1002%2Flipd.12027 https://www.ncbi.nlm.nih.gov/pubmed/29663386 https://www.proquest.com/docview/2026416302 https://www.proquest.com/docview/2718336025
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