Biosynthetic routes of molecular species of lung phosphatidylglycerol.

Okano, Goroh; Sato, Tadahiro; Akino, Toyoaki

doi:10.1620/tjem.135.265

Cited by 2 publications

(3 citation statements)

References 24 publications

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“…The dominant phospholipid is dipalmitoylphosphatidylcholine, a glycerophospholipid that was not decreased in plasma or lymphoblasts from RCDP1 patients, in our study. In contrast, we observed decreases in the levels of the major surfactant phosphatidyglycerol PG 34:1 [17] in both plasma and lympoblasts from RCDP1 subjects. Since we observed no decrements in phosphatidylinositol levels nor in the incorporation of labeled glycerol or palmitic acid into phosphatiylglycerol pools,we conclude that the de novo CDP-DAG biosynthetic pathway [23] and acylation / transacylation remodeling of phosphatidylglyverols [33] are operational in RCDP1.…”

Section: Discussioncontrasting

confidence: 70%

“…While these increases in cellular plasmalogen levels did not alter phosphatidylglycerol levels in control lymphoblasts, this treatment did increase the levels of phosphatidylglycerols (32:0 and 32:1) in RCDP1 lymphoblasts Figure 5, lower panel. These are predominant phosphatidylglycerols involved in surfactant synthesis in humans [16][17][18]. …”

Section: Ether Lipid Supplementationmentioning

confidence: 99%

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Lipidomics Analysis of Peroxisomal Disorders: Discovery of Deficits in Phosphatidyglycerol Levels in Rhizomelic Chondrodysplasia Type 1

Wood¹,

Braverman²

2014

J Data Mining Genomics Proteomics

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Objectives: Decreases in the levels of plasmalogens, have been consistently demonstrated in rhizomelic chondrodysplasia type 1 (RCDP1), a genetic disorder of peroxisomal function. However, an in-depth lipidomics analysis has not been undertaken. We undertook such an analysis. Study Design:We performed a high-resolution mass spectrometric shotgun lipidomics analyses of plasma and lymphoblasts from RCDP1 patients. Results:We report for the first time, decrements in phosphatidylglycerol levels in plasma and lymphoblasts from RCDP1 patients. Phosphatidylinositol and phosphatidylserine levels also were unaltered in plasma and lymphoblasts. These data suggested that decrements in phosphatidylglycerol were due to increased catabolism, possibly in failed cellular attempts to restore deficient plasmalogen levels. This conclusion was further supported by supplementation of RCDP1 lymphoblasts with ether lipid plasmalogen precursors that bypass dysfunctional peroxisomes. These precursors augmented cellular levels of plasmalogens in control and RCDP1 lymphoblasts but only augmented phosphatidylglycerols in RCDP1 lymphoblasts. Conclusions:Overall, our results indicate that the peroxisomal disorder, RCDP1, which is characterized by plasmalogen deficits, also possess decrements in phosphatidylglycerol levels, thereby also compromising mitochondrial function and pulmonary surfactant synthesis. Given the role pf phosphatidylglycerols in surfactant, these new data potentially explain the severe respiratory compromise in RCDP children and may add a new parameter of mitochondrial dysfunction in these patients.

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Section: Discussioncontrasting

confidence: 70%

Section: Ether Lipid Supplementationmentioning

confidence: 99%

Lipidomics Analysis of Peroxisomal Disorders: Discovery of Deficits in Phosphatidyglycerol Levels in Rhizomelic Chondrodysplasia Type 1

Wood¹,

Braverman²

2014

J Data Mining Genomics Proteomics

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“…[8,9] Interestingly, PG appears to be more susceptible than DSPC to changes in degradation and recycling. [10,11] Stable isotope tracers have proved to be a powerful, safe, and non-invasive technique to study the kinetics of different metabolic pathways without using radioactive tracers. [12] As previously published by our group, [13,14] stable isotope techniques can be successfully employed to trace DSPC kinetics in preterm infants, children and adults without interference with medical treatments.…”

mentioning

confidence: 99%

Simultaneous measurement of phosphatidylglycerol and disaturated‐phosphatidylcholine palmitate kinetics from alveolar surfactant. Study in infants with stable isotope tracer, coupled with isotope ratio mass spectrometry

et al. 2011

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Disaturated-phosphatidylcholine (DSPC) and phosphatidylglycerol (PG) are respectively the first and the third most abundant phospholipid in human alveolar surfactant. Their concentration decreases in airway surfactant of adults and infants with respiratory distress syndrome and cystic fibrosis. In this study, we used mass spectrometry (IRMS) to investigate the turnover of DSPC and PG in tracheal aspirates (TA) obtained from infants with normal or diseased lungs. We studied eight infants requiring mechanical ventilation: two with no lung disease, four with diaphragmatic hernia, one with ATP-binding cassette sub-family A member 3 heterozygote mutation and one with sepsis. Patients received deuterated water for 48 h as metabolic precursors of palmitate-DSPC and palmitate-PG. Serial TAs were obtained every 6 h for five days or until extubation. DSPC and PG were isolated from TA by column and high-performance thin layer chromatography. Deuterium enrichments of palmitate-DSPC and PG residues were measured by IRMS coupled with a gas chromatographer. Median secretion time (ST), peak time (PT) and fractional synthesis rate (FSR) were 3.7 [0.9- 13.4] h, 71.0 [52.2 - 85.2] h and 6.6 [6.3 - 11.1] %/day for DSPC and 19.3 [6.4 - 22.8] h, 49.0 [33.0 - 52.5] h and 5.8 [4.8 - 10.9] %/day for PG. This study shows that it is feasible to use deuterium derived from body water to trace simultaneously airway surfactant DSPC and PG in humans. When compared within the same patient, DSPC and PG had similar fractional synthesis rates, but PG had a shorter PT, suggesting differences in the life cycle of these essential surfactant components.

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Biosynthetic routes of molecular species of lung phosphatidylglycerol.

Cited by 2 publications

References 24 publications

Lipidomics Analysis of Peroxisomal Disorders: Discovery of Deficits in Phosphatidyglycerol Levels in Rhizomelic Chondrodysplasia Type 1

Lipidomics Analysis of Peroxisomal Disorders: Discovery of Deficits in Phosphatidyglycerol Levels in Rhizomelic Chondrodysplasia Type 1

Simultaneous measurement of phosphatidylglycerol and disaturated‐phosphatidylcholine palmitate kinetics from alveolar surfactant. Study in infants with stable isotope tracer, coupled with isotope ratio mass spectrometry

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