Full Text Journal Articles by
Author Hilde Laeremans

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Inter-laboratory analytical improvement of succinylacetone and nitisinone quantification from dried blood spot samples.

Hilde Laeremans, Charles Turner, Tommy Andersson, Jose Angel Cocho de Juan, Adam Gerrard, M Rebecca Heiner-Fokkema, Diran Herebian, Nils Janzen, Giancarlo la Marca, Mattias Rudebeck,

Background:Nitisinone is used to treat hereditary tyrosinemia type 1 (HT-1) by preventing accumulation of toxic metabolites, including succinylacetone (SA). Accurate quantification of SA during newborn screening is essential, as is quantification of both SA and nitisinone for disease monitoring and optimization of treatment. Analysis of dried blood spots (DBS) rather ... Read more >>

JIMD Rep (JIMD reports)
[2020, 53(1):90-102]

Cited: 0 times

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Parenteral hydroxocobalamin dose intensification in five patients with different types of early onset intracellular cobalamin defects: Clinical and biochemical responses.

Emmanuel Scalais, Elise Osterheld, Christine Geron, Charlotte Pierron, Ronit Chafai, Vincent Schlesser, Patricia Borde, Luc Regal, Hilde Laeremans, Koen L I van Gassen, L Bert van den Heuvel, Linda De Meirleir,

Intracellular cobalamin metabolism (ICM) defects can be present as autosomal recessive or X-linked disorders. Parenteral hydroxocobalamin (P-OHCbl) is the mainstay of therapy, but the optimal dose has not been determined. Despite early treatment, long-term complications may develop. We have analyzed the biochemical and clinical responses in five patients with early ... Read more >>

JIMD Rep (JIMD reports)
[2019, 49(1):70-79]

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Clinical implementation of gene panel testing for lysosomal storage diseases.

Alexander Gheldof, Sara Seneca, Katrien Stouffs, Willy Lissens, Anna Jansen, Hilde Laeremans, Patrick Verloo, An-Sofie Schoonjans, Marije Meuwissen, Diana Barca, Geert Martens, Linda De Meirleir,

BACKGROUND:The diagnostic workup in patients with a clinical suspicion of lysosomal storage diseases (LSD) is often difficult due to the variability in the clinical phenotype. The gold standard for diagnosis of LSDs consists of enzymatic testing. However, due to the sequential nature of this methodology and inconsistent genotype-phenotype correlations of ... Read more >>

Mol Genet Genomic Med (Molecular genetics & genomic medicine)
[2019, 7(2):e00527]

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Blocking of frizzled signaling with a homologous peptide fragment of wnt3a/wnt5a reduces infarct expansion and prevents the development of heart failure after myocardial infarction.

Hilde Laeremans, Tilman M Hackeng, Marc A M J van Zandvoort, Victor L J L Thijssen, Ben J A Janssen, Harry C J Ottenheijm, Jos F M Smits, W Matthijs Blankesteijn,

BACKGROUND: The molecular pathways that control the wound healing after myocardial infarction (MI) are not completely elucidated. One of these pathways is the Wnt/Frizzled pathway. In this study, we evaluated Frizzled as a novel therapeutic target for MI. These Frizzled proteins act as receptors for Wnt proteins and were previously ... Read more >>

Circulation (Circulation)
[2011, 124(15):1626-1635]

Cited: 65 times

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Wnt/frizzled signalling modulates the migration and differentiation of immortalized cardiac fibroblasts.

Hilde Laeremans, Sander S Rensen, Harry C J Ottenheijm, Jos F M Smits, W Matthijs Blankesteijn,

AIMS: The Wnt/frizzled (Fzd) signal transduction cascade has been implicated in the proliferation, differentiation, and migration of many cell types, but the role of this pathway in cardiac fibroblast differentiation is not known. Our lab previously showed an up-regulation of Fzd-1 and -2 expression in myofibroblasts after myocardial infarction (MI), ... Read more >>

Cardiovasc. Res. (Cardiovascular research)
[2010, 87(3):514-523]

Cited: 32 times

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Angiotensin AT4 receptor ligand interaction with cystinyl aminopeptidase and aminopeptidase N: [125I]Angiotensin IV only binds to the cystinyl aminopeptidase apo-enzyme.

Heidi Demaegdt, Pieter-Jan Lenaerts, Julie Swales, Jean-Paul De Backer, Hilde Laeremans, Minh Tam Le, Ken Kersemans, Lotte K Vogel, Yvette Michotte, Patrick Vanderheyden, Georges Vauquelin,

Due to its high affinity for [(125)I]Angiotensin IV, cystinyl aminopeptidase (CAP) has recently been assigned as the 'angiotensin AT(4) receptor'. Since the aminopeptidase N (AP-N) activity is also susceptible to inhibition by Angiotensin IV, it might represent an additional target for this peptide. Based on [(125)I]Angiotensin IV binding and catalytic ... Read more >>

Eur. J. Pharmacol. (European journal of pharmacology)
[2006, 546(1-3):19-27]

Cited: 18 times

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Metal ion modulation of cystinyl aminopeptidase.

Hilde Laeremans, Heidi Demaegdt, Jean-Paul De Backer, Minh T Le, Veerle Kersemans, Yvette Michotte, Georges Vauquelin, Patrick M L Vanderheyden,

Cystinyl aminopeptidase has one Zn2+-binding motif and is a member of the M1 aminopeptidase family. Ion modulation of its catalytic activity was studied in membranes of CHO-K1 cells (Chinese-hamster ovary K1 cells) using L-leucine-p-nitroanilide as substrate. The planar bidentate chelators 1,10-phenanthroline and 2,2'-bipyridine inhibited the activity in a concentration-dependent manner ... Read more >>

Biochem. J. (The Biochemical journal)
[2005, 390(Pt 1):351-357]

Cited: 8 times

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Synergistic modulation of cystinyl aminopeptidase by divalent cation chelators.

Heidi Demaegdt, Hilde Laeremans, Jean-Paul De Backer, Sabrina Mosselmans, Minh Tam Le, Veerle Kersemans, Yvette Michotte, Georges Vauquelin, Patrick M L Vanderheyden,

Membranes of Chinese hamster ovary (CHO-K1) cells were used to study the opposite modulation of enzyme activity and [125I]Ang IV binding to cystinyl aminopeptidase (EC 3.4.11.3) by divalent cation chelators. Whereas ethylene diamine tetraacetic acid (EDTA) or ethylene glycol-bis(2-aminoethylether)-N,N,N',N'-tetraacetic acid (EGTA) alone only slightly affected the enzyme activity, 1,10-phenanthrolin (1,10-PHE) ... Read more >>

Biochem. Pharmacol. (Biochemical pharmacology)
[2004, 68(5):893-900]

Cited: 12 times

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Endogenous cystinyl aminopeptidase in Chinese hamster ovary cells: characterization by [125I]Ang IV binding and catalytic activity.

Heidi Demaegdt, Patrick Vanderheyden, Jean-Paul De Backer, Sabrina Mosselmans, Hilde Laeremans, Minh Tam Le, Veerle Kersemans, Yvette Michotte, Georges Vauquelin,

The angiotensin II C-terminal hexapeptide fragment angiotensin IV (Ang IV) exerts central and cardiovascular effects. Cystinyl aminopeptidase (EC 3.4.11.3), a membrane-associated zinc-dependent metallopeptidase of the M1 family, has recently been found to display high affinity for Ang IV and it was proposed to represent the AT4 receptor. We present evidence ... Read more >>

Biochem. Pharmacol. (Biochemical pharmacology)
[2004, 68(5):885-892]

Cited: 29 times

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