Impaired response of fibroblasts from patients with hyperapobetalipoproteinemia to acylation-stimulating protein.
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Why the study?
Does acylation-stimulating protein (ASP) stimulate triglyceride synthesis differently in fibroblasts from patients with hyperapobetalipoproteinemia compared to normal subjects?
Population
Cultured skin fibroblasts from 31 subjects: normal subjects, patients with hyperapobetalipoproteinemia…
Comparison
Acylation-stimulating protein added to cultured… vs Comparison between different patient groups and…
Design
Preclinical
Authors
Discussion
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Implication
Reduced ASP responsiveness in hyperapoB fibroblasts leaves open a receptor defect; hypothesis-generating for targeted therapies pending in vivo confirmation.
Structured PICO
Does acylation-stimulating protein (ASP) stimulate triglyceride synthesis differently in fibroblasts from patients with hyperapobetalipoproteinemia compared to normal subjects?
P
Population
Cultured skin fibroblasts from 31 subjects: normal subjects (n=8), patients with hyperapobetalipoproteinemia (n=12), patients with familial hypercholesterolemia (n=7), and patients with hypertriglyceridemia without hyperapobetalipoproteinemia (type IV-normal B) (n=4).
I
Intervention
Acylation-stimulating protein (ASP) added to cultured skin fibroblasts in vitro.
C
Comparator
Comparison between different patient groups (normal vs. hyperapobetalipoproteinemia vs. familial hypercholesterolemia vs. type IV hypertriglyceridemia) and baseline (no ASP).
O
Outcome
Triglyceride synthesis induced by ASP and specific saturable binding of radioiodinated ASP to fibroblasts.surrogate
Fibroblasts from patients with hyperapobetalipoproteinemia demonstrate a blunted triglyceride synthesis response to acylation-stimulating protein, driven by a reduced number of specific ASP receptors.
Limitations
- Hyperapobetalipoproteinemia is a phenotype with likely multiple etiologies, not a single genotype.
- Not all hyperapoB patients had reduced ASP receptors on their fibroblasts.
- It remains to be shown that reduced ASP receptor number will be a faithful cell marker of the disorder within families with hyperapoB.
Cite This Study
Cianflone et al. (1990) studied this question.
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