What question did this study set out to answer?

To determine how lysosomal storage affects the profile and concentration of bis(monoacylglycero)phosphate across diverse lysosomal storage disorders and assess its viability as a biomarker.

March 13, 2008Biochemical Journal

Effect of lysosomal storage on bis(monoacylglycero)phosphate

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Authors

PMPeter J. MeiklePreventive Cardiology SDStephen DuplockSouth Australian Health and Medical Research Institute DBDavid BlacklockWomen's and Children's Health Network

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Implication

Comparative lipidomic analysis reveals altered bis(monoacylglycero)phosphate species in lysosomal storage disorders, indicating its utility as a disease-specific plasma biomarker.

Key Points

To determine how lysosomal storage affects the profile and concentration of bis(monoacylglycero)phosphate across diverse lysosomal storage disorders and assess its viability as a biomarker.
Assayed cultured skin fibroblasts from patients with eight lysosomal storage disorders and plasma samples from patients with one of 20 lysosomal storage disorders alongside control plasma.
Quantified individual molecular species of bis(monoacylglycero)phosphate and phosphatidylglycerol using electrospray ionization tandem mass spectrometry.
Cultured fibroblasts across all tested conditions except Fabry disease demonstrated a marked loss of polyunsaturated relative to monounsaturated bis(monoacylglycero)phosphate species, while phosphatidylglycerol species remained unchanged.
Plasma concentrations of the C(18:1)/C(18:1) bis(monoacylglycero)phosphate species were selectively elevated in disorders presenting with macrophage storage and/or hepatomegaly compared with controls, but showed no elevation in disorders with predominantly central nervous system pathology.

Cite This Study

Meikle et al. (2008) studied this question.

synapsesocial.com/papers/6a706063f44fa9f079de00de https://doi.org/10.1042/bj20071043

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Implication

Comparative lipidomic analysis reveals altered bis(monoacylglycero)phosphate species in lysosomal storage disorders, indicating its utility as a disease-specific plasma biomarker.

Effect of lysosomal storage on bis(monoacylglycero)phosphate

Discussion

Member takes

Implication

Key Points

Cite This Study

Effect of lysosomal storage on bis(monoacylglycero)phosphate

Discussion

Member takes

Implication

Key Points

Cite This Study

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