title: Analysis of the lipids of normal and Gaucher bone marrow.
abstract: Quantitative chemical shift magnetic resonance imaging (QCSI) is currently being utilized for measuring the extent of bone marrow involvement and its response to enzyme replacement therapy in patients with Gaucher's disease. Quantitation of the major lipid species in human bone marrow is required to accurately interpret QCSI data on bone marrow composition. The major lipid species in bone marrow specimens from normal individuals and from patients with type 1 Gaucher's disease were analyzed by thin-layer and high-pressure liquid chromatography. In normal marrow (N = 5), triglycerides were by far the most abundant lipid (278 +/- 70 mg/gm wet wt), with other non-polar lipids and phospholipids totaling less than 20 mg/gm wet weight. The concentration of glucocerebroside in normal marrow was 0.061 +/- 0.06 mg/gm wet weight. Gaucher marrow (N = 9) had dramatically lower triglyceride levels (51 +/- 53 mg/gm wet wt), and as expected, it had markedly elevated levels of glucocerebroside (7.1 +/- 3.4 mg/gm wet wt). The other major non-polar lipids and phospholipids were measured in selected specimens, but none were found that differed so profoundly between normal and Gaucher's disease. These data support a model of bone marrow alteration in Gaucher's disease in which triglyceride-rich adipocytes are progressively replaced by storage cells, leading to an overall reduction in total lipid content. This phenomenon provides an explanation for the changes in proton signal intensity observed in QCSI studies of Gaucher bone marrow.
authors.affiliation: Developmental and Metabolic Neurology Branch, NINDS, National Institutes of Health, Bethesda, Maryland, USA.
authors.forename: S P
authors.initials: SP
authors.lastname: Miller
authors.forename: G C
authors.initials: GC
authors.lastname: Zirzow
authors.forename: S H
authors.initials: SH
authors.lastname: Doppelt
authors.forename: R O
authors.initials: RO
authors.lastname: Brady
authors.forename: N W
authors.initials: NW
authors.lastname: Barton
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000737:chemistry*; D002648:Child; D002851:Chromatography, High Pressure Liquid; D002855:Chromatography, Thin Layer; D005260:Female; D005776:Gaucher Disease / Q000378:metabolism* / Q000473:pathology; D005963:Glucosylceramides / Q000032:analysis; D006801:Humans; D008055:Lipids / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D010743:Phospholipids / Q000032:analysis; D014280:Triglycerides / Q000032:analysis
chemical_list: D005963:Glucosylceramides; D008055:Lipids; D010743:Phospholipids; D014280:Triglycerides