Cystathionine metabolism in neuroblastoma.

Abstract

Cystathioninuria is a frequent and highly specific marker of neuroblastoma, but the etiology of this abnormality has not been well studied. To investigate this phenomenon, we analyzed 27 human neuroblastoma tissue specimens for the presence of cystathionine synthase and cystathionase. Levels of cystathionine synthase varied from undetectable to 622 pmol/mg protein, but no specimen had cystathionase measurable by rocket radioimmunoassay or catalytic assay. In addition, we assayed neuroblastoma cell lines exposed to a variety of differentiating agents: butyric acid, dimethyl sulfoxide, serum-free medium, or sodium citrate to induce differentiation. In each case we were unable to demonstrate cystathionase induction. These data are consistent with the hypothesis that neuroblastomas have a biochemical block in the transsulfuration enzymes at the level of cystathionase and that expression of cystathionine synthase in the absence of cystathionase may account for the presence of cystathioninuria in patients with neuroblastoma.

• Authors: C E Klein, B Roberts, J Holcenberg, L M Glode
• Journal: Cancer (Cancer) Vol. 62 Issue 2 Pg. 291-8 (Jul 15 1988) ISSN: 0008-543X [Print] United States
• PMID: 3383129 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Cystathionine
• Cystathionine beta-Synthase
• Cystathionine gamma-Lyase

Topics

• Cell Differentiation
• Cell Line
• Cystathionine (metabolism)
• Cystathionine beta-Synthase (metabolism)
• Cystathionine gamma-Lyase (metabolism)
• Humans
• Liver (enzymology)
• Lymphocytes (metabolism)
• Neuroblastoma (metabolism)