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Gene Review

SMS  -  spermine synthase

Homo sapiens

Synonyms: MRSR, SPMSY, SRS, SpS, Spermidine aminopropyltransferase, ...
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Disease relevance of SMS


Psychiatry related information on SMS

  • The intraperitoneal administration of SMS resulted in a dose-dependent and selective increase of paradoxical sleep (SP) [3].
  • The battery of psychomotor tests included peak velocity of saccadic eye movements (SEM), a Sternberg memory scanning and choice reaction time test (SMS-CRT) and critical flicker fusion frequency (CFFF) [4].
  • Some results of speech perception tests from the first two SMSP users are presented, in which scores for the recognition of vowels, consonants, and words all showed significant increases over the corresponding MSP scores [5].
  • We examined the psychometric properties of the Self-Regulation Scale (SRS; Schwarzer, Diehl, & Schmitz, 1999), a measure of attention control in goal pursuit, in 2 independent studies [6].
  • We found support for the criterion validity of the SRS in terms of positive correlations with measures of general and domain-specific self-efficacy, proactive coping, and positive affect and in terms of negative correlations with depressive symptoms and negative affect [6].

High impact information on SMS

  • SRS is genetically heterogenous with maternal uniparental disomy with respect to chromosome 7 occurring in approximately 10% of affected individuals [7].
  • Two other lines (SMS-MSN and SMS-SAN) were established from different patients before any therapy was given [8].
  • Moreover, these preparations were able to generate significant quantities of SRS-A (32 +/- 7 x 10(-17) LTD mole-equivalents/mast cell) at all stages of purification, indicating that a secondary cell is not necessary for the antigen-induced release of SRS [9].
  • If a primary gastrinoma cannot be identified by SRS or STIR-MRI, endoscopic ultrasonography should be undertaken because duodenal gastrinomas are often minute and multicentric [10].
  • A similar strategy applies for insulinomas because up to 40% cannot be located by SRS and the majority are located in the pancreatic head [10].

Chemical compound and disease context of SMS

  • The effects on gallbladder motility of long term treatment with the somatostatin analog SMS 201-995 (SMS) were studied in five patients with acromegaly treated for 6-32 months with 200-300 micrograms SMS daily [11].
  • In the patient with paraneoplastic hypercortisolism, SMS was associated with a progressive decrease, though not the normalization, of UFC and of ACTH and cortisol levels [12].
  • Patients used SMS to transmit data such as blood glucose levels and body weight to a server [13].
  • The data emphasize that SMS exhibits pro-apoptotic and anti-proliferative effects, which in proper dose combinations might enhance the effects of 5-FU on human colorectal cancer cells expressing mp53 [14].
  • This finding suggests that independent pathways mediate the growth-inhibitory effects of tamoxifen and SMS, and that different pancreatic cancers may respond to the two agents differently, some with inhibition, some not [15].

Biological context of SMS

  • We performed FISH with additional chromosome 17 probes, SMS (Smith-Magenis syndrome critical region) and RARA (retinoic acid receptor), on 7 cases with unusual Her-2/CEP17 (chromosome 17 centromere control probe) results to assess whether different measurements of chromosome 17 copy number might clarify the Her-2/neu amplicon status [16].
  • SpS consists of 11 exons spread over 54 kb [17].
  • Strikingly, human, mouse and Caenorhabditis elegans genomes each contain at least two different SM synthase (SMS) genes [18].
  • There are several pseudogenes of SMS / Sms in man and mouse [19].
  • The study of SMS, a rare disease, has resulted in a better understanding of a more common disorder, IDDM, and has allowed investigators to gain insights into the molecular mechanisms of autoimmunity [20].

Anatomical context of SMS

  • Many unanswered questions remain, such as the specific site of disease activity in SMS, both at the bedside (cortex, brain stem, or spinal cord) and at the bench (neuronal cytoplasma or synapse) [20].
  • SMS completely suppressed the postprandial gallbladder contraction, despite a blunted, though still statistically significant, increase in plasma CCK from 1.6 +/- 0.2 pmol/L to an average of 3.7 +/- 1.7 pmol/L (P less than 0.01) [11].
  • While somatostatin-(1-14) is completely inactive on monocyte chemotaxis, the synthetic analog SMS 201995 is extremely potent [21].
  • The growth of 1/3 SSR positive SCLC cell lines was significantly inhibited by the long-acting somatostatin analogue octreotide (SMS 201-995, Sandostatin) 10(-9) M [22].
  • We previously demonstrated that SMS inhibits T cells acting on the CD28 rather than the CD3-mediated signal in exactly the same way as does IL-10 [23].

Associations of SMS with chemical compounds

  • Sequence comparisons between human spermine synthase and spermidine synthases from bacterial and mammalian sources revealed a nearly complete lack of similarity between the primary structures of these two enzymes catalyzing almost identical reactions [24].
  • SMS may be useful as an antidiarrhoeal drug in patients with high output jejuno- or ileostomies, but in patients who need permanent parenteral nutrition the effect is too small to significantly alter management [25].
  • SMS 201-995 (SMS) is a long-acting analog of somatostatin [26].
  • After a control day, 50 micrograms SMS were injected sc, and plasma GH and insulin and blood glucose levels were measured at multiple intervals for 24 h [27].
  • Circulating GH was not altered by SMS or the dopamine agonist mesulergine in one patient, but the combination of both substances (50 micrograms SMS, sc, and 0.5 mg mesulergine, orally) reduced GH to below 50% of basal [27].
  • The spermine synthase.5'-methylthioadenosine structure provides a plausible explanation for the potent inhibition of the reaction by this product and the stronger inhibition of spermine synthase compared with spermidine synthase [28].

Physical interactions of SMS

  • We describe novel application of SMS/RARA FISH probes for assessing cases with complex Her-2/CEP17 FISH patterns [16].
  • Furthermore, SRIF-binding sites were undetectable in a patient which did not respond to SMS 201995 therapy [29].

Regulatory relationships of SMS

  • CCK-induced gall bladder contraction was significantly inhibited by a single dose of 25 micrograms SMS 201-995 but not after 7 days of pretreatment with the somatostatin analogue [30].

Other interactions of SMS

  • It is shown that SRS develops before SBS and suddenly decays around the peak of the pump, as the IAW reaches saturation [31].
  • Although the Her-2/CEP17 ratio scores were within normal range (<2.0), the Her-2/SMS or Her-2/RARA ratio revealed amplification of Her-2/neu in 5 of 7 cases [16].
  • X-linked spermine synthase gene (SMS) defect: the first polyamine deficiency syndrome [1].
  • The apparent lack of an overall similarity may indicate that spermine synthase, the enzyme found only in eukaryotes, and spermidine synthase with more universal distribution, although functionally closely related, have evolved separately [24].
  • Somatostatin analog SMS 201995 inhibits proliferation in human leukemia T-cell line: relevance of the adenylyl cyclase stimulation [32].

Analytical, diagnostic and therapeutic context of SMS

  • Molecular cloning of a cDNA encoding human spermine synthase [24].
  • Sequence analysis of 139 kb in Xp22.1 containing spermine synthase and the 5' region of PEX [17].
  • PARTICIPANTS: Nonfederal, postresident patient care physicians who provided more than 47 000 responses to SMS surveys between 1983 and 1994 [33].
  • The LIM 2412 xenografts in the SMS-treated animals were 473.3 +/- 99.9 (SD) versus 838.1 +/- 111.3 mm3 in control (P less than 0.05) after 20 days [34].
  • In immunoblot analyses, an SMS serum--but only 1/10 randomly selected IDDM sera--recognized the blotted rGAD64 without relation to immunoprecipitation titers [35].


  1. X-linked spermine synthase gene (SMS) defect: the first polyamine deficiency syndrome. Cason, A.L., Ikeguchi, Y., Skinner, C., Wood, T.C., Holden, K.R., Lubs, H.A., Martinez, F., Simensen, R.J., Stevenson, R.E., Pegg, A.E., Schwartz, C.E. Eur. J. Hum. Genet. (2003) [Pubmed]
  2. Treatment of the malignant carcinoid syndrome. Evaluation of a long-acting somatostatin analogue. Kvols, L.K., Moertel, C.G., O'Connell, M.J., Schutt, A.J., Rubin, J., Hahn, R.G. N. Engl. J. Med. (1986) [Pubmed]
  3. The somatostatin analogue SMS 201-995 promotes paradoxical sleep in aged rats. Danguir, J. Neurobiol. Aging (1989) [Pubmed]
  4. The comparison of the effects of multi and single doses of buspirone, chlordiazepoxide and hydroxyzine on psychomotor function and EEG. Blom, M.W., Bartel, P.R., Sommers, D.K., Van der Meyden, C.H., Becker, P.J. Fundamental & clinical pharmacology. (1992) [Pubmed]
  5. A new portable sound processor for the University of Melbourne/Nucleus Limited multielectrode cochlear implant. McDermott, H.J., McKay, C.M., Vandali, A.E. The Journal of the Acoustical Society of America. (1992) [Pubmed]
  6. Assessing attention control in goal pursuit: a component of dispositional self-regulation. Diehl, M., Semegon, A.B., Schwarzer, R. Journal of personality assessment. (2006) [Pubmed]
  7. Epimutation of the telomeric imprinting center region on chromosome 11p15 in Silver-Russell syndrome. Gicquel, C., Rossignol, S., Cabrol, S., Houang, M., Steunou, V., Barbu, V., Danton, F., Thibaud, N., Le Merrer, M., Burglen, L., Bertrand, A.M., Netchine, I., Le Bouc, Y. Nat. Genet. (2005) [Pubmed]
  8. Characterization of human neuroblastoma cell lines established before and after therapy. Reynolds, C.P., Biedler, J.L., Spengler, B.A., Reynolds, D.A., Ross, R.A., Frenkel, E.P., Smith, R.G. J. Natl. Cancer Inst. (1986) [Pubmed]
  9. Generation of leukotrienes by purified human lung mast cells. MacGlashan, D.W., Schleimer, R.P., Peters, S.P., Schulman, E.S., Adams, G.K., Newball, H.H., Lichtenstein, L.M. J. Clin. Invest. (1982) [Pubmed]
  10. Approaches to the diagnosis of gut neuroendocrine tumors: the last word (today). Modlin, I.M., Tang, L.H. Gastroenterology (1997) [Pubmed]
  11. Postprandial gallbladder motility during long term treatment with the long-acting somatostatin analog SMS 201-995 in acromegaly. van Liessum, P.A., Hopman, W.P., Pieters, G.F., Jansen, J.B., Smals, A.G., Rosenbusch, G., Kloppenborg, P.W., Lamers, C.B. J. Clin. Endocrinol. Metab. (1989) [Pubmed]
  12. Treatment of Cushing's syndrome with the long-acting somatostatin analogue SMS 201-995 (sandostatin). Invitti, C., de Martin, M., Brunani, A., Piolini, M., Cavagnini, F. Clin. Endocrinol. (Oxf) (1990) [Pubmed]
  13. Mobile phone text messaging in the management of diabetes. Ferrer-Roca, O., Cárdenas, A., Diaz-Cardama, A., Pulido, P. Journal of telemedicine and telecare. (2004) [Pubmed]
  14. SMS 201-995 enhances S-phase block induced by 5-fluorouracil in a human colorectal cancer cell line. Massari, D., Trobonjac, Z., Rukavina, D., Radosević-Stasić, B. Anticancer Drugs (2005) [Pubmed]
  15. Effect of somatostatin and tamoxifen on the growth of human pancreatic cancers in nude mice. Poston, G.J., Townsend, C.M., Rajaraman, S., Thompson, J.C., Singh, P. Pancreas (1990) [Pubmed]
  16. Evaluation of her-2/neu status in carcinomas with amplified chromosome 17 centromere locus. Troxell, M.L., Bangs, C.D., Lawce, H.J., Galperin, I.B., Baiyee, D., West, R.B., Olson, S.B., Cherry, A.M. Am. J. Clin. Pathol. (2006) [Pubmed]
  17. Sequence analysis of 139 kb in Xp22.1 containing spermine synthase and the 5' region of PEX. Grieff, M., Whyte, M.P., Thakker, R.V., Mazzarella, R. Genomics (1997) [Pubmed]
  18. Identification of a family of animal sphingomyelin synthases. Huitema, K., van den Dikkenberg, J., Brouwers, J.F., Holthuis, J.C. EMBO J. (2004) [Pubmed]
  19. Spermine deficiency in Gy mice caused by deletion of the spermine synthase gene. Lorenz, B., Francis, F., Gempel, K., Böddrich, A., Josten, M., Schmahl, W., Schmidt, J., Lehrach, H., Meitinger, T., Strom, T.M. Hum. Mol. Genet. (1998) [Pubmed]
  20. Stiff-man syndrome: from the bedside to the bench. Helfgott, S.M. Arthritis Rheum. (1999) [Pubmed]
  21. Human monocyte chemotactic activity of calcitonin and somatostatin related peptides: modulation by chronic peptide treatment. Sacerdote, P., Bianchi, M., Panerai, A.E. J. Clin. Endocrinol. Metab. (1990) [Pubmed]
  22. Experimental and clinical studies with somatostatin analogue octreotide in small cell lung cancer. Macaulay, V.M., Smith, I.E., Everard, M.J., Teale, J.D., Reubi, J.C., Millar, J.L. Br. J. Cancer (1991) [Pubmed]
  23. Regulation of human peripheral blood lymphocytes IL-10 BY SMS 201-995. Casnici, C., Lattuada, D., Franco, P., Cattaneo, L., Marelli, O. J. Neuroimmunol. (2004) [Pubmed]
  24. Molecular cloning of a cDNA encoding human spermine synthase. Korhonen, V.P., Halmekytö, M., Kauppinen, L., Myöhänen, S., Wahlfors, J., Keinänen, T., Hyvönen, T., Alhonen, L., Eloranta, T., Jänne, J. DNA Cell Biol. (1995) [Pubmed]
  25. Effect of a long acting somatostatin analogue SMS 201-995 on jejunostomy effluents in patients with severe short bowel syndrome. Ladefoged, K., Christensen, K.C., Hegnhøj, J., Jarnum, S. Gut (1989) [Pubmed]
  26. The response of serum growth hormone levels to the long-acting somatostatin analog SMS 201-995 in acromegaly. Comi, R.J., Gorden, P. J. Clin. Endocrinol. Metab. (1987) [Pubmed]
  27. The somatostatin analog SMS 201-995 induces long-acting inhibition of growth hormone secretion without rebound hypersecretion in acromegalic patients. Lamberts, S.W., Oosterom, R., Neufeld, M., del Pozo, E. J. Clin. Endocrinol. Metab. (1985) [Pubmed]
  28. Crystal structure of human spermine synthase: implications of substrate binding and catalytic mechanism. Wu, H., Min, J., Zeng, H., McCloskey, D.E., Ikeguchi, Y., Loppnau, P., Michael, A.J., Pegg, A.E., Plotnikov, A.N. J. Biol. Chem. (2008) [Pubmed]
  29. Somatostatin receptors in brain and pituitary. Epelbaum, J., Agid, F., Agid, Y., Beaudet, A., Bertrand, P., Enjalbert, A., Heidet, V., Kordon, C., Krantic, S., Léonard, J.F. Horm. Res. (1989) [Pubmed]
  30. Effect of the somatostatin analogue sandostatin (SMS 201-995) on gastrointestinal, pancreatic and biliary function and hormone release in normal men. Lembcke, B., Creutzfeldt, W., Schleser, S., Ebert, R., Shaw, C., Koop, I. Digestion (1987) [Pubmed]
  31. Transient development of backward stimulated Raman and Brillouin scattering on a picosecond time scale measured by subpicosecond thomson diagnostic. Rousseaux, C., Gremillet, L., Casanova, M., Loiseau, P., Rabec Le Gloahec, M., Baton, S.D., Amiranoff, F., Adam, J.C., Héron, A. Phys. Rev. Lett. (2006) [Pubmed]
  32. Somatostatin analog SMS 201995 inhibits proliferation in human leukemia T-cell line: relevance of the adenylyl cyclase stimulation. Giannetti, N., Enjalbert, A., Krantic, S. J. Cell. Biochem. (2000) [Pubmed]
  33. Current trends in physicians' practice arrangements. From owners to employees. Kletke, P.R., Emmons, D.W., Gillis, K.D. JAMA (1996) [Pubmed]
  34. SMS 201.995 inhibits in vitro and in vivo growth of human colon cancer. Dy, D.Y., Whitehead, R.H., Morris, D.L. Cancer Res. (1992) [Pubmed]
  35. Recombinant glutamic acid decarboxylase (representing the single isoform expressed in human islets) detects IDDM-associated 64,000-M(r) autoantibodies. Karlsen, A.E., Hagopian, W.A., Petersen, J.S., Boel, E., Dyrberg, T., Grubin, C.E., Michelsen, B.K., Madsen, O.D., Lernmark, A. Diabetes (1992) [Pubmed]
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