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Article ; Online: Plasma oxalate and glycolate concentrations in dialysis patients with and without primary hyperoxaluria type 1.

Metry, Elisabeth L / Garrelfs, Sander F / Peters-Sengers, Hessel / Vaz, Frederic M / Bijlsma, Joost A / Neradova, Aegida / Oosterveld, Michiel J S / Groothoff, Jaap W

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

2023 Volume 38, Issue 7, Page(s) 1773–1775

MeSH term(s)	Humans ; Oxalates ; Renal Dialysis ; Hyperoxaluria, Primary ; Glycolates ; Hyperoxaluria
Chemical Substances	Oxalates ; glycolic acid (0WT12SX38S) ; Glycolates
Language	English
Publishing date	2023-03-03
Publishing country	England
Document type	Journal Article
ZDB-ID	90594-x
ISSN	1460-2385 ; 0931-0509
ISSN (online)	1460-2385
ISSN	0931-0509
DOI	10.1093/ndt/gfad049
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Zs.A 2198: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 329: Show issues

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Article ; Online: Ophthalmic acid is a glutathione regulating tripeptide.

Schomakers, Bauke V / Jillings, Sonia L / van Weeghel, Michel / Vaz, Frédéric M / Salomons, Gajja S / Janssens, Georges E / Houtkooper, Riekelt H

The FEBS journal

2024

Abstract: Since its discovery in 1958 in the lens of cows, ophthalmic acid (OPH) has stood in the shadow of its anti-oxidant analog: glutathione (GSH). Lacking the thiol group that gives GSH many of its important properties, ophthalmic acid's function has remained ...

Abstract	Since its discovery in 1958 in the lens of cows, ophthalmic acid (OPH) has stood in the shadow of its anti-oxidant analog: glutathione (GSH). Lacking the thiol group that gives GSH many of its important properties, ophthalmic acid's function has remained elusive, and it has been widely presumed to be an accidental product of the same enzymes. In this review, we compile evidence demonstrating that OPH is a ubiquitous metabolite found in bacteria, plants, fungi, and animals, produced through several layers of metabolic regulation. We discuss the limitations of the oft-repeated suggestions that aberrations in OPH levels should solely indicate GSH deficiency or oxidative stress. Finally, we discuss the available literature and suggest OPH's role in metabolism as a GSH-regulating tripeptide; controlling both cellular and organelle influx and efflux of GSH, as well as modulating GSH-dependent reactions and signaling. Ultimately, we hope that this review reinvigorates and directs more research into this versatile metabolite.
Language	English
Publishing date	2024-01-20
Publishing country	England
Document type	Journal Article
ZDB-ID	2173655-8
ISSN	1742-4658 ; 1742-464X
ISSN (online)	1742-4658
ISSN	1742-464X
DOI	10.1111/febs.17061
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Article ; Online: Plasma Lipidomic Profiles in cART-Treated Adolescents with Perinatally Acquired HIV Compared to Matched Controls.

van der Post, Julie / Guerra, Thiara E J / van den Hof, Malon / Vaz, Frédéric M / Pajkrt, Dasja / van Genderen, Jason G

Viruses

2024 Volume 16, Issue 4

Abstract: Children with perinatally acquired human immunodeficiency virus (PHIV) are growing into adulthood with HIV and treatment-associated comorbidities, such as dyslipidemia and insulin resistance. HIV is identified as independent risk factor for ... ...

Abstract	Children with perinatally acquired human immunodeficiency virus (PHIV) are growing into adulthood with HIV and treatment-associated comorbidities, such as dyslipidemia and insulin resistance. HIV is identified as independent risk factor for cardiovascular disease (CVD). The hypothesis behind increased CVD risk associated with HIV includes vascular inflammation, dyslipidemia and combination antiretroviral therapy (cART) metabolomic toxicity. To investigate differences in lipid profiles and pathophysiological mechanisms of CVD risk in adolescents with PHIV, we compared the plasma lipidome of PHIV adolescents and HIV-negative controls. We additionally investigated the influence of current cART regimens and increased lipoprotein(a) (Lp(a)) levels on the plasma lipidome. We included 20 PHIV-infected adolescents and 20 HIV-negative controls matched for age, sex, ethnic origin and socio-economic status. Plasma lipidome was measured using Thermo Scientific Ultimate 3000 binary high-performance liquid chromatography (HPLC)-mass spectrometry. We evaluated the plasma lipidome in PHIV adolescents using different cART regimens (including those known to be associated with lipid alterations). The median age was 17.5 years (15.5-20.7) and 16.5 years (15.7-19.8) for PHIV adolescents and controls, respectively. Of PHIV adolescents, 45% used a non-nucleotide reverse transcriptase inhibitor (NNRTI)-based (25%) or protease inhibitor (PI)-based (20%) cART regimen. In this pilot study, we observed no significant differences between lipidomic profiles between PHIV adolescents and controls. We observed no differences in the plasma lipidome in participants with increased versus normal Lp(a) levels. Different cART regimens appear to influence chain length differences in the plasma lipidome of PHIV adolescents; however, the significance and causality of this observation remains undetermined. Further research on the influence of cART on lipid composition could further identify these alterations.
MeSH term(s)	Humans ; Adolescent ; HIV Infections/drug therapy ; Female ; Male ; Lipidomics ; Lipids/blood ; Young Adult ; Anti-HIV Agents/therapeutic use ; Case-Control Studies ; Anti-Retroviral Agents/therapeutic use ; Infectious Disease Transmission, Vertical ; Cardiovascular Diseases
Chemical Substances	Lipids ; Anti-HIV Agents ; Anti-Retroviral Agents
Language	English
Publishing date	2024-04-09
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2516098-9
ISSN	1999-4915 ; 1999-4915
ISSN (online)	1999-4915
ISSN	1999-4915
DOI	10.3390/v16040580
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Article ; Online: Barth syndrome and the many fascinating aspects of cardiolipin.

Vaz, Frédéric M / Wanders, Ronald J A / Vernon, Hilary

Journal of inherited metabolic disease

2021 Volume 45, Issue 1, Page(s) 1–2

MeSH term(s)	Acyltransferases/genetics ; Barth Syndrome/genetics ; Barth Syndrome/metabolism ; Cardiolipins/metabolism ; Humans
Chemical Substances	Cardiolipins ; Acyltransferases (EC 2.3.-) ; TAFAZZIN protein, human (EC 2.3.1.-)
Language	English
Publishing date	2021-12-14
Publishing country	United States
Document type	Editorial
ZDB-ID	438341-2
ISSN	1573-2665 ; 0141-8955
ISSN (online)	1573-2665
ISSN	0141-8955
DOI	10.1002/jimd.12460
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Article ; Online: The plasma kynurenine-to-tryptophan ratio as a biomarker of tuberculosis disease in people living with HIV on antiretroviral therapy: an exploratory nested case-control study.

Gatechompol, Sivaporn / Lutter, René / Vaz, Frédéric M / Ubolyam, Sasiwimol / Avihingsanon, Anchalee / Kerr, Stephen J / van Leth, Frank / Cobelens, Frank

BMC infectious diseases

2024 Volume 24, Issue 1, Page(s) 372

Abstract: Background: Non-sputum-based tests are needed to predict or diagnose tuberculosis (TB) disease in people living with HIV (PWH). The enzyme indoleamine 2, 3-dioxygenase-1 (IDO1) is expressed in tuberculoid granuloma and catabolizes tryptophan (Trp) to ... ...

Abstract	Background: Non-sputum-based tests are needed to predict or diagnose tuberculosis (TB) disease in people living with HIV (PWH). The enzyme indoleamine 2, 3-dioxygenase-1 (IDO1) is expressed in tuberculoid granuloma and catabolizes tryptophan (Trp) to kynurenine (Kyn). IDO1 activity compromises innate and adaptive immune responses, promoting mycobacterial survival. The plasma Kyn-to-Trp (K/T) ratio is a potential TB diagnostic and/or predictive biomarker in PWH on long-term antiretroviral therapy (ART). Methods: We compared plasma K/T ratios in samples from PWH, who were followed up prospectively and developed TB disease after ART initiation. Controls were matched for age and duration of ART. Kyn and Trp were measured at 3 timepoints; at TB diagnosis, 6 months before TB diagnosis and 6 months after TB diagnosis, using ultra performance liquid chromatography combined with mass spectrometry. Results: The K/T ratios were higher for patients with TB disease at time of diagnosis (median, 0.086; IQR, 0.069-0.123) compared to controls (0.055; IQR 0.045-0.064; p = 0.006), but not before or after TB diagnosis. K/T ratios significantly declined after successful TB treatment, but increased upon treatment failure. The K/T ratios showed a parabolic correlation with CD4 cell counts in participants with TB (p = 0.005), but there was no correlation in controls. Conclusions: The plasma K/T ratio helped identify TB disease and may serve as an adjunctive biomarker for for monitoring TB treatment in PWH. Validation studies to ascertain these findings and evaluate the optimum cut-off for diagnosis of TB disease in PWH should be undertaken in well-designed prospective cohorts. Trial registration: ClinicalTrials.gov Identifier: NCT00411983.
MeSH term(s)	Humans ; Tryptophan ; Kynurenine ; Prospective Studies ; Case-Control Studies ; HIV Infections/complications ; HIV Infections/drug therapy ; Tuberculosis/diagnosis ; Tuberculosis/drug therapy ; Biomarkers ; Indoleamine-Pyrrole 2,3,-Dioxygenase
Chemical Substances	Tryptophan (8DUH1N11BX) ; Kynurenine (343-65-7) ; Biomarkers ; Indoleamine-Pyrrole 2,3,-Dioxygenase
Language	English
Publishing date	2024-04-02
Publishing country	England
Document type	Journal Article
ZDB-ID	2041550-3
ISSN	1471-2334 ; 1471-2334
ISSN (online)	1471-2334
ISSN	1471-2334
DOI	10.1186/s12879-024-09258-4
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Article ; Online: Long-term Efficacy and Safety of Elamipretide in Patients with Barth Syndrome: 168-Week Open-label Extension Results of TAZPOWER.

Thompson, William R / Manuel, Ryan / Abbruscato, Anthony / Carr, Jim / Campbell, John / Hornby, Brittany / Vaz, Frédéric M / Vernon, Hilary J

Genetics in medicine : official journal of the American College of Medical Genetics

2024 , Page(s) 101138

Abstract: Purpose: Evaluate long-term efficacy and safety of elamipretide during the open-label extension (OLE) of the TAZPOWER trial in individuals with Barth syndrome (BTHS) .: Methods: TAZPOWER was a 28-week randomized, double-blind, placebo-controlled ... ...

Abstract	Purpose: Evaluate long-term efficacy and safety of elamipretide during the open-label extension (OLE) of the TAZPOWER trial in individuals with Barth syndrome (BTHS) . Methods: TAZPOWER was a 28-week randomized, double-blind, placebo-controlled trial followed by a 168-week OLE. Patients entering the OLE continued elamipretide 40mg subcutaneous daily. OLE primary endpoints were safety and tolerability; secondary endpoints included change from baseline in the 6-minute walk test (6MWT) and BarTH Syndrome Symptom Assessment (BTHS-SA) Total Fatigue. Muscle strength, physician- and patient-assessed outcomes, echocardiographic parameters, and biomarkers, including cardiolipin (CL) and monolysocardiolipin (MLCL), were assessed. Results: Ten patients entered the OLE; 8 reached the Week 168 visit. Elamipretide was well tolerated, with injection site reactions being the most common adverse events. Significant improvements from OLE baseline on 6MWT occurred at all OLE timepoints (cumulative 96.1 meters of improvement [Week 168, p=0.003]). Mean BTHS-SA Total Fatigue scores were below baseline (improved) at all OLE timepoints. 3-D left ventricular stroke, end-diastolic, and end-systolic volumes improved, showing significant trends for improvement from baseline to Week 168. MLCL/CL values showed improvement, correlating to important clinical outcomes. Conclusion: Elamipretide was associated with sustained long-term tolerability and efficacy, with improvements in functional assessments and cardiac function in BTHS.
Language	English
Publishing date	2024-04-07
Publishing country	United States
Document type	Journal Article
ZDB-ID	1455352-1
ISSN	1530-0366 ; 1098-3600
ISSN (online)	1530-0366
ISSN	1098-3600
DOI	10.1016/j.gim.2024.101138
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Zs.A 5059: Show issues

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Article ; Online: Glycolate oxidase inhibition by lumasiran varies between patients with primary hyperoxaluria type 1.

Garrelfs, Sander F / Metry, Elisabeth L / van Harskamp, Dewi / Vaz, Frederic M / van den Akker, Chris H P / Schierbeek, Henk / Groothoff, Jaap W / Oosterveld, Michiel J S

Kidney international

2023 Volume 103, Issue 5, Page(s) 990–993

MeSH term(s)	Humans ; Hyperoxaluria, Primary/diagnosis ; Hyperoxaluria, Primary/drug therapy ; Hyperoxaluria, Primary/genetics ; Alcohol Oxidoreductases ; Hyperoxaluria ; Oxalates
Chemical Substances	glycollate oxidase (EC 1.1.3.15) ; lumasiran (RZT8C352O1) ; Alcohol Oxidoreductases (EC 1.1.-) ; Oxalates
Language	English
Publishing date	2023-03-04
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2023.01.029
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Zs.A 797: Show issues

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Article ; Online: A novel homozygous missense variant in ARSK causes MPS X, a new subtype of mucopolysaccharidosis

Miao Sun / Cornelia K. Kaminsky / Philip Deppe / Mai-Britt Ilse / Frédéric M. Vaz / Barbara Plecko / Torben Lübke / Linda M. Randolph

Genes and Diseases, Vol 11, Iss 3, Pp 101025- (2024)

2024

Keywords	Medicine (General) ; R5-920 ; Genetics ; QH426-470
Language	English
Publishing date	2024-05-01T00:00:00Z
Publisher	KeAi Communications Co., Ltd.
Document type	Article ; Online
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Article: Fatty Acid Oxidation in Peroxisomes: Enzymology, Metabolic Crosstalk with Other Organelles and Peroxisomal Disorders.

Wanders, Ronald J A / Vaz, Frédéric M / Waterham, Hans R / Ferdinandusse, Sacha

Advances in experimental medicine and biology

2021 Volume 1299, Page(s) 55–70

Abstract: Peroxisomes play a central role in metabolism as exemplified by the fact that many genetic disorders in humans have been identified through the years in which there is an impairment in one or more of these peroxisomal functions, in most cases associated ... ...

Abstract	Peroxisomes play a central role in metabolism as exemplified by the fact that many genetic disorders in humans have been identified through the years in which there is an impairment in one or more of these peroxisomal functions, in most cases associated with severe clinical signs and symptoms. One of the key functions of peroxisomes is the β-oxidation of fatty acids which differs from the oxidation of fatty acids in mitochondria in many respects which includes the different substrate specificities of the two organelles. Whereas mitochondria are the main site of oxidation of medium-and long-chain fatty acids, peroxisomes catalyse the β-oxidation of a distinct set of fatty acids, including very-long-chain fatty acids, pristanic acid and the bile acid intermediates di- and trihydroxycholestanoic acid. Peroxisomes require the functional alliance with multiple subcellular organelles to fulfil their role in metabolism. Indeed, peroxisomes require the functional interaction with lysosomes, lipid droplets and the endoplasmic reticulum, since these organelles provide the substrates oxidized in peroxisomes. On the other hand, since peroxisomes lack a citric acid cycle as well as respiratory chain, oxidation of the end-products of peroxisomal fatty acid oxidation notably acetyl-CoA, and different medium-chain acyl-CoAs, to CO
MeSH term(s)	Fatty Acids/metabolism ; Humans ; Lipid Metabolism ; Oxidation-Reduction ; Peroxisomal Disorders/enzymology ; Peroxisomal Disorders/metabolism ; Peroxisomes/enzymology ; Peroxisomes/metabolism
Chemical Substances	Fatty Acids
Language	English
Publishing date	2021-01-08
Publishing country	United States
Document type	Journal Article ; Review
ISSN	2214-8019 ; 0065-2598
ISSN (online)	2214-8019
ISSN	0065-2598
DOI	10.1007/978-3-030-60204-8_5
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Article ; Online: Inherited disorders of complex lipid metabolism: A clinical review.

Xiao, Changrui / Rossignol, Francis / Vaz, Frédéric M / Ferreira, Carlos R

Journal of inherited metabolic disease

2021 Volume 44, Issue 4, Page(s) 809–825

Abstract: Over 80 human diseases have been attributed to defects in complex lipid metabolism. A majority of them have been reported recently in the setting of rapid advances in genomic technology and their increased use in clinical settings. Lipids are ubiquitous ... ...

Abstract	Over 80 human diseases have been attributed to defects in complex lipid metabolism. A majority of them have been reported recently in the setting of rapid advances in genomic technology and their increased use in clinical settings. Lipids are ubiquitous in human biology and play roles in many cellular and intercellular processes. While inborn errors in lipid metabolism can affect every organ system with many examples of genetic heterogeneity and pleiotropy, the clinical manifestations of many of these disorders can be explained based on the disruption of the metabolic pathway involved. In this review, we will discuss the physiological function of major pathways in complex lipid metabolism, including nonlysosomal sphingolipid metabolism, acylceramide metabolism, de novo phospholipid synthesis, phospholipid remodeling, phosphatidylinositol metabolism, mitochondrial cardiolipin synthesis and remodeling, and ether lipid metabolism as well as common clinical phenotypes associated with each.
MeSH term(s)	Cardiolipins/biosynthesis ; Cardiolipins/chemistry ; Homeostasis ; Humans ; Lipid Metabolism ; Lipids/biosynthesis ; Lipids/chemistry ; Metabolic Networks and Pathways/physiology ; Mitochondria/metabolism ; Mitochondria/pathology ; Phenotype ; Phosphatidylinositols/biosynthesis ; Phosphatidylinositols/chemistry ; Phospholipids/biosynthesis ; Phospholipids/chemistry ; Sphingolipids/biosynthesis ; Sphingolipids/chemistry
Chemical Substances	Cardiolipins ; Lipids ; Phosphatidylinositols ; Phospholipids ; Sphingolipids
Language	English
Publishing date	2021-03-02
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	438341-2
ISSN	1573-2665 ; 0141-8955
ISSN (online)	1573-2665
ISSN	0141-8955
DOI	10.1002/jimd.12369
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