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Article ; Online: In vitro alternative for reactogenicity assessment of outer membrane vesicle based vaccines.

Molenaar-de Backer, Marijke W A / Doodeman, Paulien / Rezai, Fereshte / Verhagen, Lisa M / van der Ark, Arno / Plagmeijer, Els M / Metz, Bernard / van Vlies, Naomi / Ophorst, Olga / Raeven, René H M

Scientific reports

2023 Volume 13, Issue 1, Page(s) 12675

Abstract: Intrinsic or added immune activating molecules are key for most vaccines to provide desired immunity profiles but may increase systemic reactogenicity. Regulatory agencies require rabbit pyrogen testing (RPT) for demonstration of vaccine reactogenicity. ... ...

Abstract	Intrinsic or added immune activating molecules are key for most vaccines to provide desired immunity profiles but may increase systemic reactogenicity. Regulatory agencies require rabbit pyrogen testing (RPT) for demonstration of vaccine reactogenicity. Recently, the monocyte activation test (MAT) gained popularity as in vitro alternative, yet this assay was primarily designed to test pyrogen-free products. The aim was to adjust the MAT to enable testing of pyrogen containing vaccines in an early stage of development where no reference batch is yet available. The MAT and RPT were compared for assessing unknown safety profiles of pertussis outer membrane vesicle (OMV) vaccine candidates to those of Bexsero as surrogate reference vaccine. Pertussis OMVs with wild-type LPS predominantly activated TLR2 and TLR4 and were more reactogenic than Bexsero. However, this reactogenicity profile for pertussis OMVs could be equalized or drastically reduced compared to Bexsero or a whole-cell pertussis vaccine, respectively by dose changing, modifying the LPS, intranasal administration, or a combination of these. Importantly, except for LPS modified products, reactogenicity profiles obtained with the RPT and MAT were comparable. Overall, we demonstrated that this pertussis OMV vaccine candidate has an acceptable safety profile. Furthermore, the MAT proved its applicability to assess reactogenicity levels of pyrogen containing vaccines at multiple stages of vaccine development and could eventually replace rabbit pyrogen testing.
MeSH term(s)	Animals ; Rabbits ; Lipopolysaccharides/pharmacology ; Whooping Cough ; Pyrogens ; Monocytes ; Biological Assay
Chemical Substances	Lipopolysaccharides ; Pyrogens
Language	English
Publishing date	2023-08-04
Publishing country	England
Document type	Journal Article
ZDB-ID	2615211-3
ISSN	2045-2322 ; 2045-2322
ISSN (online)	2045-2322
ISSN	2045-2322
DOI	10.1038/s41598-023-39908-7
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Article ; Online: Slc22a5 haploinsufficiency does not aggravate the phenotype of the long-chain acyl-CoA dehydrogenase KO mouse.

Ranea-Robles, Pablo / Yu, Chunli / van Vlies, Naomi / Vaz, Frédéric M / Houten, Sander M

Journal of inherited metabolic disease

2019 Volume 43, Issue 3, Page(s) 486–495

Abstract: Secondary carnitine deficiency is commonly observed in inherited metabolic diseases characterised by the accumulation of acylcarnitines such as mitochondrial fatty acid oxidation (FAO) disorders. It is currently unclear if carnitine deficiency and/or ... ...

Abstract	Secondary carnitine deficiency is commonly observed in inherited metabolic diseases characterised by the accumulation of acylcarnitines such as mitochondrial fatty acid oxidation (FAO) disorders. It is currently unclear if carnitine deficiency and/or acylcarnitine accumulation play a role in the pathophysiology of FAO disorders. The long-chain acyl-CoA dehydrogenase (LCAD) KO mouse is a model for long-chain FAO disorders and is characterised by decreased levels of tissue and plasma free carnitine. Tissue levels of carnitine are controlled by SLC22A5, the plasmalemmal carnitine transporter. Here, we have further decreased carnitine availability in the LCAD KO mouse through a genetic intervention by introducing one defective Slc22a5 allele (jvs). Slc22a5 haploinsufficiency decreased free carnitine levels in liver, kidney, and heart of LCAD KO animals. The resulting decrease in the tissue long-chain acylcarnitines levels had a similar magnitude as the decrease in free carnitine. Levels of cardiac deoxycarnitine, a carnitine biosynthesis intermediate, were elevated due to Slc22a5 haploinsufficiency in LCAD KO mice. A similar increase in heart and muscle deoxycarnitine was observed in an independent experiment using Slc22a5
MeSH term(s)	Acyl-CoA Dehydrogenase, Long-Chain/deficiency ; Acyl-CoA Dehydrogenase, Long-Chain/genetics ; Acyl-CoA Dehydrogenase, Long-Chain/metabolism ; Animals ; Cardiomyopathies ; Carnitine/analogs & derivatives ; Carnitine/deficiency ; Carnitine/pharmacology ; Disease Models, Animal ; Fatty Acids/metabolism ; Female ; Haploinsufficiency ; Hyperammonemia ; Lipid Metabolism/drug effects ; Liver/metabolism ; Male ; Mice ; Mice, Knockout ; Muscular Diseases ; Myocardium/metabolism ; Phenotype ; Solute Carrier Family 22 Member 5/genetics ; Solute Carrier Family 22 Member 5/metabolism
Chemical Substances	Fatty Acids ; Slc22a5 protein, mouse ; Solute Carrier Family 22 Member 5 ; acylcarnitine ; Acyl-CoA Dehydrogenase, Long-Chain (EC 1.3.8.8) ; Carnitine (S7UI8SM58A)
Language	English
Publishing date	2019-12-23
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural
ZDB-ID	438341-2
ISSN	1573-2665 ; 0141-8955
ISSN (online)	1573-2665
ISSN	0141-8955
DOI	10.1002/jimd.12204
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Article: Perturbed body fluid distribution and osmoregulation in response to high salt intake in patients with hereditary multiple exostoses.

Oppelaar, Jetta J / Rorije, Nienke M G / Olde Engberink, Rik H G / Chahid, Youssef / van Vlies, Naomi / Verberne, Hein J / van den Born, Bert-Jan H / Vogt, Liffert

Molecular genetics and metabolism reports

2021 Volume 29, Page(s) 100797

Abstract: Background: Hereditary Multiple Exostoses (HME) is a rare autosomal disorder characterized by the presence of multiple exostoses (osteochondromas) caused by a heterozygous loss of function mutation in : Methods: We performed a randomized cross-over ... ...

Abstract	Background: Hereditary Multiple Exostoses (HME) is a rare autosomal disorder characterized by the presence of multiple exostoses (osteochondromas) caused by a heterozygous loss of function mutation in Methods: We performed a randomized cross-over study in 7 male HME patients and 12 healthy controls, matched for age, BMI, blood pressure and renal function. All subjects followed both an 8-day low sodium diet (LSD, <50 mmol/d) and high sodium diet (HSD, >200 mmol/d) in randomized order. After each diet, blood and urine samples were collected. Body fluid compartment measurements were performed by using the distribution curve of iohexol and Results: In HME patients, HSD resulted in significant increase of intracellular fluid volume (ICFV) (1.2 L, Conclusion: HME patients show altered body fluid distribution and osmoregulation after HSD compared to controls. Our results might indicate reduced interstitial sodium accumulation capacity in HME, leading to ICFV increase. Therefore, this study provides additional support that HS is crucial for maintaining constancy of the internal environment.
Language	English
Publishing date	2021-11-05
Publishing country	United States
Document type	Journal Article
ZDB-ID	2821908-9
ISSN	2214-4269
ISSN	2214-4269
DOI	10.1016/j.ymgmr.2021.100797
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Article ; Online: Supplementing pregnant mice with a specific mixture of nondigestible oligosaccharides reduces symptoms of allergic asthma in male offspring.

Hogenkamp, Astrid / Thijssen, Suzan / van Vlies, Naomi / Garssen, Johan

The Journal of nutrition

2014 Volume 145, Issue 3, Page(s) 640–646

Abstract: Background: Previously, maternal supplementation with short-chain galacto- and long-chain fructo-oligosaccharides (scGOS/lcFOS; ratio 9:1) was shown to affect maternal and fetal immune status in mice.: Objective: This study was designed to test the ... ...

Abstract	Background: Previously, maternal supplementation with short-chain galacto- and long-chain fructo-oligosaccharides (scGOS/lcFOS; ratio 9:1) was shown to affect maternal and fetal immune status in mice. Objective: This study was designed to test the long-term effects of supplementation of mice with scGOS/lcFOS before and during pregnancy on the immune response in the offspring, using an ovalbumin (OVA)-induced model for experimental allergic asthma. Methods: Female Balb/c mice were fed a control diet or a diet supplemented with 3% scGOS/lcFOS and mated to C57BL/6 males. All dams were fed the control diet after delivery. At 6 wk, male offspring received an intraperitoneal injection of aluminum hydroxide and OVA (control and scGOS/lcFOS group) or saline (sham group). The acute allergic skin response (ASR) after intradermal challenge with OVA or saline was measured at 8 wk. After 3 airway challenges with nebulized OVA or saline, lung function was measured. Results: The scGOS/lcFOS group had a significantly lower acute ASR (85 ± 9 μm) than the control group (124 ± 9 μm; P = 0.01). Lower lung resistance from a response to methacholine challenge was seen in the scGOS/lcFOS group. OVA-specific immunoglobulin (Ig)E concentrations in the control group [93 ± 45 arbitrary unit (AU)] and the scGOS/lcFOS group (67 ± 45 AU) were higher than in the sham group (11 ± 2 AU). OVA specific IgG2a concentrations in the scGOS/lcFOS (146 ± 24 AU) were higher than in the sham group (2 ± 0.3 AU) and control group (18 ± 3.5 AU; P < 0.05). Finally, the scGOS/lcFOS group had a higher percentage of regulatory T cells (1.11% ± 0.07%) than the sham group (0.14% ± 0.03%) and the control group (0.11% ± 0.02%; P < 0.05). Conclusion: Maternal supplementation of mice with scGOS/lcFOS during pregnancy leads to a significant decrease in allergic symptoms in the offspring.
MeSH term(s)	Animals ; Asthma/prevention & control ; Dermatitis, Atopic/prevention & control ; Dietary Supplements ; Female ; Immunoglobulin E/blood ; Immunoglobulin G/blood ; Male ; Mice ; Mice, Inbred BALB C ; Mice, Inbred C57BL ; Oligosaccharides/administration & dosage ; Ovalbumin/adverse effects ; Prebiotics ; Pregnancy
Chemical Substances	Immunoglobulin G ; Oligosaccharides ; Prebiotics ; Immunoglobulin E (37341-29-0) ; Ovalbumin (9006-59-1)
Language	English
Publishing date	2014-12-24
Publishing country	United States
Document type	Journal Article
ZDB-ID	218373-0
ISSN	1541-6100 ; 0022-3166
ISSN (online)	1541-6100
ISSN	0022-3166
DOI	10.3945/jn.114.197707
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Article ; Online: Prediction of phenotypic severity in mucopolysaccharidosis type IIIA.

Knottnerus, Suzan J G / Nijmeijer, Stephanie C M / IJlst, Lodewijk / Te Brinke, Heleen / van Vlies, Naomi / Wijburg, Frits A

Annals of neurology

2017 Volume 82, Issue 5, Page(s) 686–696

Abstract: Objective: Mucopolysaccharidosis IIIA or Sanfilippo disease type A is a progressive neurodegenerative disorder presenting in early childhood, caused by an inherited deficiency of the lysosomal hydrolase sulfamidase. New missense mutations, for which ... ...

Abstract	Objective: Mucopolysaccharidosis IIIA or Sanfilippo disease type A is a progressive neurodegenerative disorder presenting in early childhood, caused by an inherited deficiency of the lysosomal hydrolase sulfamidase. New missense mutations, for which genotype-phenotype correlations are currently unknown, are frequently reported, hampering early prediction of phenotypic severity and efficacy assessment of new disease-modifying treatments. We aimed to design a method to determine phenotypic severity early in the disease course. Methods: Fifty-three patients were included for whom skin fibroblasts and data on disease course and mutation analysis were available. Patients were phenotypically characterized on clinical data as rapidly progressing or slowly progressing. Sulfamidase activity was measured in fibroblasts cultured at 37 °C and at 30 °C. Results: Sulfamidase activity in fibroblasts from patients homozygous or compound heterozygous for a combination of known severe mutations remained below the limit of quantification under both culture conditions. In contrast, sulfamidase activity in fibroblasts from patients homozygous or compound heterozygous for a known mild mutation increased above the limit of quantification when cultured at 30 °C. With division on the basis of the patients' phenotype, fibroblasts from slowly progressing patients could be separated from rapidly progressing patients by increase in sulfamidase activity when cultured at 30 °C (p < 0.001, sensitivity = 96%, specificity = 93%). Interpretation: Phenotypic severity strongly correlates with the potential to increase sulfamidase activity in fibroblasts cultured at 30 °C, allowing reliable distinction between patients with rapidly progressing or slowly progressing phenotypes. This method may provide an essential tool for assessment of treatment effects and for health care and life planning decisions. Ann Neurol 2017;82:686-696.
Language	English
Publishing date	2017-11
Publishing country	United States
Document type	Journal Article
ZDB-ID	80362-5
ISSN	1531-8249 ; 0364-5134
ISSN (online)	1531-8249
ISSN	0364-5134
DOI	10.1002/ana.25069
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Zs.A 1370: 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)
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Article ; Online: Abnormal sodium and water homeostasis in mice with defective heparan sulfate polymerization.

Olde Engberink, Rik H G / de Vos, Judith / van Weert, Angela / Zhang, Yahua / van Vlies, Naomi / van den Born, Bert-Jan H / Titze, Jens M / van Bavel, Ed / Vogt, Liffert

PloS one

2019 Volume 14, Issue 7, Page(s) e0220333

Abstract: Glycosaminoglycans in the skin interstitium and endothelial surface layer have been shown to be involved in local sodium accumulation without commensurate water retention. Dysfunction of heparan sulfate glycosaminoglycans may therefore disrupt sodium and ...

Abstract	Glycosaminoglycans in the skin interstitium and endothelial surface layer have been shown to be involved in local sodium accumulation without commensurate water retention. Dysfunction of heparan sulfate glycosaminoglycans may therefore disrupt sodium and water homeostasis. In this study, we investigated the effects of combined heterozygous loss of heparan sulfate polymerization genes (exostosin glycosyltransferase 1 and 2; Ext1+/-Ext2+/-) on sodium and water homeostasis. Sodium storage capacity was decreased in Ext1+/-Ext2+/- mice as reflected by a 77% reduction in endothelial surface layer thickness and a lower skin sodium-to-glycosaminoglycan ratio. Also, these mice were characterized by a higher heart rate, increased fluid intake, increased plasma osmolality and a decreased skin water and sodium content, suggesting volume depletion. Upon chronic high sodium intake, the initial volume depletion was restored but no blood pressure increase was observed. Acute hypertonic saline infusion resulted in a distinct blood pressure response: we observed a significant 15% decrease in control mice whereas blood pressure did not change in Ext1+/-Ext2+/- mice. This differential blood pressure response may be explained by the reduced capacity for sodium storage and/or the impaired vasodilation response, as measured by wire myography, which was observed in Ext1+/-Ext2+/- mice. Together, these data demonstrate that defective heparan sulfate glycosaminoglycan synthesis leads to abnormal sodium and water homeostasis and an abnormal response to sodium loading, most likely caused by inadequate capacity for local sodium storage.
MeSH term(s)	Animals ; Blood Pressure ; Electrolytes/blood ; Female ; Heart Rate ; Heparitin Sulfate/chemistry ; Heterozygote ; Male ; Mice ; Mice, Inbred C57BL ; Myography ; N-Acetylglucosaminyltransferases/genetics ; N-Acetylglucosaminyltransferases/metabolism ; Polymerization ; Skin/chemistry ; Skin/metabolism ; Sodium/metabolism ; Water/metabolism
Chemical Substances	Electrolytes ; Water (059QF0KO0R) ; Heparitin Sulfate (9050-30-0) ; Sodium (9NEZ333N27) ; N-Acetylglucosaminyltransferases (EC 2.4.1.-) ; exostosin-1 (EC 2.4.1.224) ; exostosin-2 (EC 2.4.1.224)
Language	English
Publishing date	2019-07-31
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1932-6203
ISSN (online)	1932-6203
DOI	10.1371/journal.pone.0220333
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Article ; Online: Genistein increases glycosaminoglycan levels in mucopolysaccharidosis type I cell models.

Kingma, Sandra D K / Wagemans, Tom / IJlst, Lodewijk / Wijburg, Frits A / van Vlies, Naomi

Journal of inherited metabolic disease

2014 Volume 37, Issue 5, Page(s) 813–821

Abstract: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder characterized by diminished degradation of the glycosaminoglycans (GAGs) heparan sulfate and dermatan sulfate, which results in the accumulation of these GAGs and subsequent cellular ... ...

Abstract	Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder characterized by diminished degradation of the glycosaminoglycans (GAGs) heparan sulfate and dermatan sulfate, which results in the accumulation of these GAGs and subsequent cellular dysfunction. Patients present with a variety of symptoms, including severe skeletal disease. Genistein has been shown previously to inhibit GAG synthesis in MPS fibroblasts, presumably through inhibition of tyrosine kinase activity of the epidermal growth factor receptor (EGFR). To determine the potentials of genistein for the treatment of skeletal disease, MPS I fibroblasts were induced into chondrocytes and osteoblasts and treated with genistein. Surprisingly, whereas tyrosine phosphorylation levels (as a measure for tyrosine kinase inhibition) were decreased in all treated cell lines, there was a 1.3 and 1.6 fold increase in GAG levels in MPS I chondrocytes and fibroblast, respectively (p < 0.05). Sulfate incorporation in treated MPS I fibroblasts was 2.6 fold increased (p < 0.05), indicating increased GAG synthesis despite tyrosine kinase inhibition. This suggests that GAG synthesis is not exclusively regulated through the tyrosine kinase activity of the EGFR. We hypothesize that the differences in outcomes between studies on the effect of genistein in MPS are caused by the different effects of genistein on different growth factor signaling pathways, which regulate GAG synthesis. More studies are needed to elucidate the precise signaling pathways which are affected by genistein and alter GAG metabolism in order to evaluate the therapeutic potential of genistein for MPS patients.
MeSH term(s)	Cell Differentiation/drug effects ; Cell Line ; Cell Survival/drug effects ; Chondrocytes/drug effects ; Chondrocytes/metabolism ; Enzyme Inhibitors/pharmacology ; Genistein/pharmacology ; Glycosaminoglycans/metabolism ; Humans ; Isoflavones/pharmacology ; Mucopolysaccharidosis I/genetics ; Mucopolysaccharidosis I/metabolism ; Osteoblasts/drug effects ; Osteoblasts/metabolism ; Sulfates/metabolism
Chemical Substances	Enzyme Inhibitors ; Glycosaminoglycans ; Isoflavones ; Sulfates ; Genistein (DH2M523P0H)
Language	English
Publishing date	2014-04-04
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	438341-2
ISSN	1573-2665 ; 0141-8955
ISSN (online)	1573-2665
ISSN	0141-8955
DOI	10.1007/s10545-014-9703-x
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Article: The Role of Virulence Proteins in Protection Conferred by

Raeven, René H M / van Vlies, Naomi / Salverda, Merijn L M / van der Maas, Larissa / Uittenbogaard, Joost P / Bindels, Tim H E / Rigters, Jolanda / Verhagen, Lisa M / Kruijer, Sabine / van Riet, Elly / Metz, Bernard / van der Ark, Arno A J

Vaccines

2020 Volume 8, Issue 3

Abstract: The limited protective immunity induced by acellular pertussis vaccines demands development of novel vaccines that induce broader and longer-lived immunity. In this study, we investigated the protective capacity of outer membrane vesicle pertussis ... ...

Abstract	The limited protective immunity induced by acellular pertussis vaccines demands development of novel vaccines that induce broader and longer-lived immunity. In this study, we investigated the protective capacity of outer membrane vesicle pertussis vaccines (omvPV) with different antigenic composition in mice to gain insight into which antigens contribute to protection. We showed that total depletion of virulence factors (
Language	English
Publishing date	2020-07-30
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2703319-3
ISSN	2076-393X
ISSN	2076-393X
DOI	10.3390/vaccines8030429
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Article ; Online: Microvascular Permeability after an Acute and Chronic Salt Load in Healthy Subjects: A Randomized Open-label Crossover Intervention Study.

Rorije, Nienke M G / Olde Engberink, Rik H G / Chahid, Youssef / van Vlies, Naomi / van Straalen, Jan P / van den Born, Bert-Jan H / Verberne, Hein J / Vogt, Liffert

Anesthesiology

2018 Volume 128, Issue 2, Page(s) 352–360

Abstract: Background: Sodium-induced microcirculatory changes, endothelial surface layer alterations in particular, may play an important role in sodium-mediated blood pressure elevation. However, effects of acute and chronic sodium loading on the endothelial ... ...

Abstract	Background: Sodium-induced microcirculatory changes, endothelial surface layer alterations in particular, may play an important role in sodium-mediated blood pressure elevation. However, effects of acute and chronic sodium loading on the endothelial surface layer and microcirculation in humans have not been established. The objective of this study was to assess sodium-induced changes in blood pressure and body weight as primary outcomes and also in microvascular permeability, sublingual microcirculatory dimensions, and urinary glycosaminoglycan excretion in healthy subjects. Methods: Twelve normotensive males followed both a low-sodium diet (less than 50 mmol/day) and a high-sodium diet (more than 200 mmol/day) for eight days in randomized order, separated by a crossover period. After the low-sodium diet, hypertonic saline (5 mmol sodium/liter body water) was administered intravenously in 30 min. Results: Both sodium interventions did not change blood pressure. Body weight increased with 2.5 (95% CI, 1.7 to 3.2) kg (P < 0.001) after dietary sodium loading. Acute intravenous sodium loading resulted in increased transcapillary escape rate of I-labeled albumin (2.7 [0.1 to 5.3] % cpm · g · h; P = 0.04), whereas chronic dietary sodium loading did not affect transcapillary escape rate of I-labeled albumin (-0.03 [-3.3 to 3.2] % cpm · g · h; P = 1.00), despite similar increases of plasma sodium and osmolality. Acute intravenous sodium loading coincided with significantly increased plasma volume, as assessed by the distribution volume of albumin, and significantly decreased urinary excretion of heparan sulfate and chondroitin sulfate. These changes were not observed after dietary sodium loading. Conclusions: Our results suggest that intravenous sodium loading has direct adverse effects on the endothelial surface layer, independent of blood pressure.
MeSH term(s)	Adolescent ; Adult ; Blood Pressure/drug effects ; Body Weight/drug effects ; Capillary Permeability/drug effects ; Cross-Over Studies ; Glycosaminoglycans/urine ; Humans ; Male ; Microcirculation/drug effects ; Saline Solution, Hypertonic/administration & dosage ; Sodium, Dietary/administration & dosage ; Sodium, Dietary/pharmacology ; Sodium, Dietary/urine ; Young Adult
Chemical Substances	Glycosaminoglycans ; Saline Solution, Hypertonic ; Sodium, Dietary
Language	English
Publishing date	2018
Publishing country	United States
Document type	Journal Article ; Randomized Controlled Trial ; Research Support, Non-U.S. Gov't
ZDB-ID	269-0
ISSN	1528-1175 ; 0003-3022
ISSN (online)	1528-1175
ISSN	0003-3022
DOI	10.1097/ALN.0000000000001989
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Article: Adverse Effects of Genistein in a Mucopolysaccharidosis Type I Mouse Model.

Kingma, Sandra D K / Wagemans, Tom / IJlst, Lodewijk / Seppen, Jurgen / Gijbels, Marion J J / Wijburg, Frits A / van Vlies, Naomi

JIMD reports

2015 Volume 23, Page(s) 77–83

Abstract: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder characterized by diminished degradation of the glycosaminoglycans heparan sulfate (HS) and dermatan sulfate (DS). Patients present with a variety of symptoms, including severe skeletal ... ...

Abstract	Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder characterized by diminished degradation of the glycosaminoglycans heparan sulfate (HS) and dermatan sulfate (DS). Patients present with a variety of symptoms, including severe skeletal disease. Current therapeutic strategies have only limited effects on bone disease. The isoflavone genistein has been studied as a potential therapy for the mucopolysaccharidoses because of its putative ability to inhibit GAG synthesis and subsequent accumulation. Cell, animal, and clinical studies, however, showed variable outcomes. To determine the effects of genistein on MPS I-related bone disease, wild-type (WT) and MPS I mice were fed a genistein-supplemented diet (corresponding to a dose of approximately 160 mg/kg/day) for 8 weeks. HS and DS levels in bone and plasma remained unchanged after genistein supplementation, while liver HS levels were decreased in genistein-fed MPS I mice as compared to untreated MPS I mice. Unexpectedly, genistein-fed mice exhibited significantly decreased body length and femur length. In addition, 60% of genistein-fed MPS I mice developed a scrotal hernia and/or scrotal hydrocele, manifestations, which were absent in WT or untreated MPS I mice. In contrast to studies in MPS III mice, our study in MPS I mice demonstraes no beneficial but even potential adverse effects of genistein supplementation. Our results urge for a cautious approach on the use of genistein, at least in patients with MPS I.
Language	English
Publishing date	2015-04-09
Publishing country	United States
Document type	Journal Article
ZDB-ID	2672872-2
ISSN	2192-8312 ; 2192-8304
ISSN (online)	2192-8312
ISSN	2192-8304
DOI	10.1007/8904_2015_432
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