Targeting glucosylceramide synthase synergizes with C6-ceramide nanoliposomes to induce apoptosis in natural killer cell leukemia

Leuk Lymphoma. 2013 Jun;54(6):1288-96. doi: 10.3109/10428194.2012.752485. Epub 2012 Dec 31.

Abstract

Abstract Natural killer (NK) cell leukemia is characterized by clonal expansion of CD3 - NK cells and comprises both chronic and aggressive forms. Currently no effective treatment exists, thus providing a need for identification of novel therapeutics. Lipidomic studies revealed a dysregulated sphingolipid metabolism as evidenced by decreased levels of overall ceramide species and increased levels of cerebrosides in leukemic NK cells, concomitant with increased glucosylceramide synthase (GCS) expression. GCS, a key enzyme of this pathway, neutralizes pro-apoptotic ceramide by transfer of a uridine diphosphate (UDP)-glucose. Thus, we treated both rat and human leukemic NK cells in combination with: (1) exogenous C6-ceramide nanoliposomes in order to target mitochondria and increase physiological pro-apoptotic levels of long chain ceramide, and (2) 1-phenyl-2-palmitoylamino-3-morpholino-1-propanol (PPMP), an inhibitor of GCS. Co-administration of C6-ceramide nanoliposomes and PPMP elicited an increase in endogenous long-chain ceramide species, which led to cellular apoptosis in a synergistic manner via the mitochondrial intrinsic cell death pathway in leukemic NK cells.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antineoplastic Agents / administration & dosage*
  • Antineoplastic Agents / metabolism
  • Apoptosis / drug effects*
  • Cell Line, Tumor
  • Cell Survival / drug effects
  • Ceramides / administration & dosage*
  • Ceramides / metabolism
  • Dose-Response Relationship, Drug
  • Drug Combinations
  • Glucosyltransferases / antagonists & inhibitors
  • Glucosyltransferases / genetics
  • Glucosyltransferases / metabolism*
  • Humans
  • Inhibitor of Apoptosis Proteins / metabolism
  • Leukemia, Large Granular Lymphocytic / genetics
  • Leukemia, Large Granular Lymphocytic / metabolism*
  • Liposomes
  • Membrane Potential, Mitochondrial / drug effects
  • Morpholines / pharmacology
  • Myeloid Cell Leukemia Sequence 1 Protein / metabolism
  • Reactive Oxygen Species / metabolism
  • Signal Transduction / drug effects
  • Sphingolipids / pharmacology
  • Survivin

Substances

  • 1-phenyl-2-palmitoylamino-3-morpholino-1-propanol
  • Antineoplastic Agents
  • BIRC5 protein, human
  • Ceramides
  • Drug Combinations
  • Inhibitor of Apoptosis Proteins
  • Liposomes
  • MCL1 protein, human
  • Morpholines
  • Myeloid Cell Leukemia Sequence 1 Protein
  • Reactive Oxygen Species
  • Sphingolipids
  • Survivin
  • Glucosyltransferases
  • ceramide glucosyltransferase