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Plasma inducible degrader of the LDLR, soluble low-density lipoprotein receptor, and proprotein convertase subtilisin/kexin type 9 levels as potential biomarkers of familial hypercholesterolemia in children
Abstract
Background Familial hypercholesterolemia (FH) in children is under-detected. Plasma biomarkers associated with low-density lipoprotein receptor (LDLR) function could help identifying FH children. Objectives We aim to assess the clinical value of inducible degrader of the LDLR (IDOL), soluble LDLR (sLDLR), and proprotein convertase subtilisin/kexin type 9 (PCSK9) plasma concentrations in children with FH compared with control children (CCh). Methods This was a cross-sectional study performed in a Lipid Unit from a University hospital. The participants were 177 children distributed into FH (n = 77) and CCh (n = 100). Main outcomes were changes in IDOL, sLDLR, and PCSK9 plasma concentrations between children groups; secondary outcomes were the association between IDOL, sLDLR, and PCSK9 and lipid profile determined by 2-dimensional nuclear magnetic resonance. Results The IDOL levels were higher in FH compared with CCh (P = .007). The PCSK9 levels were elevated in FH (P Conclusions The IDOL and PCSK9 plasma levels are significantly higher in FH children. Interestingly, sLDLR was associated with atherogenic dyslipidemia components. IDOL concentrations show a robust association with Lp(a) levels. To study the role of plasma biomarkers associated with LDLR expression in FH is warranted.
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Plasma levels of sLDLR were positively associated with non-HDL-C and small LDL numbers in FH children.
“Recently, Girona et al reported that plasma levels of sLDLR were positively associated with non-HDL-C and small LDL numbers in FH children31.”
From Membrane type 1 matrix metalloproteinase promotes LDL receptor shedding and accelerates the development of atherosclerosis (2021) · cites this paper as [31]
Serum sLDLR levels have been positively correlated with high plasma triglycerides and LDL levels.
“This mechanism may explain why serum sLDLR levels have been positively correlated with high plasma triglycerides and LDL levels [97,98].”
From The low-density lipoprotein receptor: Emerging post-transcriptional regulatory mechanisms (2025) · cites this paper as [97]
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Alabi, A., Xia, X.-D., Gu, H.-M., Wang, F., Deng, S.-J., Yang, N., Adijiang, A., Douglas, D. N., Kneteman, N. M., Xue, Y., et al. (2021) · Nature Communications · doi:10.1038/s41467-021-22167-3 · Cited by 63
The low-density lipoprotein receptor: Emerging post-transcriptional regulatory mechanisms
Ndoj, K., Meurs, A., Papaioannou, D., Bjune, K., & Zelcer, N. (2025) · Atherosclerosis · doi:10.1016/j.atherosclerosis.2024.119082 · Cited by 17
Metadata
- Authors
- Girona, J., Rodríguez-Borjabad, C., Ibarretxe, D., Heras, M., Amigo, N., Feliu, A., Masana, L., Plana, N., Amigó, E., Andrés, P., Barrio, M., Bilbao, J. Á., Bosch, M., Cabedo, J. L., Calvo, J., Campillo, C., Castejón, E., Castillejo, G., Castro, M., Cliville, R., De Gotardo, E., Domènech, V., Domínguez, D., Escolà, M., Fernández, M., García, J., Girona, R., Guijarro, E., Gutiérrez, M. A., Iglesias, D., Salsas, J. M., Luque, V., Machado, P., Maixé, J., Mallafré, M., Martin, R., Jiménez, M., Monne, R., Morillo, S., Naranjo, À., Pérez, C., Planelles, M., Querol, C., Rabadà, M. J., Remedi, A., Riquelme, C., Rodríguez, N., Rosell, L., Salvado, M., Salvador, O., Santos, A., Sanz, N., Segura, S., Subirana, G., Tarrades, P., Vendrell, M., Vilella, M., & Zabala, E.
- Venue
- Journal of Clinical Lipidology
- Publisher
- Elsevier BV
- Published
- 2018-01
- Volume
- 12
- Issue
- 1
- Pages
- 211-218
- ISSN
- 1933-2874
- Cited by
- 22
- References
- 2
- Language
- en
- Funders
- European Regional Development Fund
- Fundació la Marató de TV3
- Society for Economic Anthropology
- Centro de Investigación Biomédica en Red Diabetes y Enfermedades Metabólicas Asociadas
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