alexa Lipidomics Analysis of Peroxisomal Disorders: Discovery of Deficits in Phosphatidyglycerol Levels in Rhizomelic Chondrodysplasia Type 1 | OMICS International | Abstract
ISSN: 2153-0602

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Special Issue Article

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

Paul L Wood1* and Nancy E Braverman2,3
1Metabolomics Unit, Dept. of Physiology and Pharmacology, DeBusk College of Osteopathic Medicine, Lincoln Memorial University, 6965 Cumberland Gap Pkwy., Harrogate, TN 37752, Canada
2Department of Human Genetics, McGill University, Quebec, Canada
3Department of Pediatrics, Montreal Children's Hospital Montreal, Quebec, Canada
Corresponding Author : Paul L Wood
Dept. of Physiology and Pharmacology
DeBusk College of Osteopathic Medicine
Lincoln Memorial University, 6965 Cumberland Gap Pkwy
Harrogate, TN 37752, Canada
Tel: 423-869-6666
Fax: 423-869-7174
E-mail: [email protected]
Received May 08, 2014; Accepted June 28, 2014; Published June 30, 2014
Citation: Wood Pl, Braverman NE (2014) Lipidomics Analysis of Peroxisomal Disorders: Discovery of Deficits in Phosphatidyglycerol Levels in Rhizomelic Chondrodysplasia Type 1. J Data Mining Genomics Proteomics S1:001. doi:10.4172/2153-0602.1000S1-001
Copyright: © 2014 Wood PL et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

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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