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Roelof Ottenhoff

Explore the profile of Roelof Ottenhoff including associated specialties, affiliations and a list of published articles. Areas
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Articles 52
Citations 1428
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Recent Articles
1.
Liefhebber J, Brasser G, Spronck E, Ottenhoff R, Paerels L, Ferraz M, et al.
Mol Ther Methods Clin Dev . 2024 Dec; 32(4):101375. PMID: 39687734
We developed a novel adeno-associated virus 5 gene therapy (AAV5-GLA) expressing human alpha-galactosidase A (GLA) under the control of a novel, small and strong, liver-restricted promoter. We assessed the preclinical...
2.
Hendrix S, Tan J, Ndoj K, Kingma J, Valiloo M, Zijlstra L, et al.
Mol Cell Biol . 2024 May; 44(4):123-137. PMID: 38747374
SREBP transcription factors are central regulators of lipid metabolism. Their proteolytic activation requires ER to the Golgi translocation and subsequent cleavage by site-1-protease (S1P). Produced as a proprotein, S1P undergoes...
3.
Hendrix S, Kingma J, Ottenhoff R, Valiloo M, Svecla M, Zijlstra L, et al.
Nat Commun . 2023 Aug; 14(1):5181. PMID: 37626055
The sterol regulatory element binding proteins (SREBPs) are transcription factors that govern cholesterol and fatty acid metabolism. We recently identified SPRING as a post-transcriptional regulator of SREBP activation. Constitutive or...
4.
van Wouw S, van den Berg M, El Ouraoui M, Meurs A, Kingma J, Ottenhoff R, et al.
J Lipid Res . 2023 Jan; 64(2):100325. PMID: 36592658
Lysoplasmalogens are a class of vinyl ether bioactive lipids that have a central role in plasmalogen metabolism and membrane fluidity. The liver X receptor (LXR) transcription factors are important determinants...
5.
Clemente-Olivo M, Habibe J, Vos M, Ottenhoff R, Jongejan A, Herrema H, et al.
Metabolism . 2021 Jun; 121:154815. PMID: 34119536
Objective: Four-and-a-Half-LIM-domain-protein 2 (FHL2) modulates multiple signal transduction pathways but has not been implicated in obesity or energy metabolism. In humans, methylation and expression of the FHL2 gene increases with...
6.
van der Lienden M, Aten J, Marques A, Waas I, Larsen P, Claessen N, et al.
Int J Mol Sci . 2021 Apr; 22(5). PMID: 33802460
The lysosomal storage disease Niemann-Pick type C (NPC) is caused by impaired cholesterol efflux from lysosomes, which is accompanied by secondary lysosomal accumulation of sphingomyelin and glucosylceramide (GlcCer). Similar to...
7.
Boer D, Mirzaian M, Ferraz M, Zwiers K, Baks M, Hazeu M, et al.
J Lipid Res . 2020 Dec; 62:100018. PMID: 33361282
Deficiency of glucocerebrosidase (GBA), a lysosomal β-glucosidase, causes Gaucher disease. The enzyme hydrolyzes β-glucosidic substrates and transglucosylates cholesterol to cholesterol-β-glucoside. Here we show that recombinant human GBA also cleaves β-xylosides...
8.
van Loon N, van Wouw S, Ottenhoff R, Nelson J, Kingma J, Scheij S, et al.
Atherosclerosis . 2020 Nov; 315:1-9. PMID: 33190106
Background And Aims: Cholesterol metabolism is tightly regulated by transcriptional and post-transcriptional mechanisms. Accordingly, dysregulation of cholesterol metabolism is a major risk factor for the development of coronary artery disease...
9.
Loregger A, Raaben M, Nieuwenhuis J, Tan J, Jae L, van den Hengel L, et al.
Nat Commun . 2020 Mar; 11(1):1128. PMID: 32111832
The sterol-regulatory element binding proteins (SREBP) are central transcriptional regulators of lipid metabolism. Using haploid genetic screens we identify the SREBP Regulating Gene (SPRING/C12ORF49) as a determinant of the SREBP...
10.
Herrera Moro Chao D, Wang Y, Foppen E, Ottenhoff R, van Roomen C, Parlevliet E, et al.
Diabetes . 2019 Oct; 68(12):2223-2234. PMID: 31578192
Obesity is taking on worldwide epidemic proportions, yet effective pharmacological agents with long-term efficacy remain unavailable. Previously, we designed the iminosugar N-adamantine-methyloxypentyl-deoxynojirimycin (AMP-DNM), which potently improves glucose homeostasis by lowering...