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Gene Review

CSE  -  choreoathetosis/spasticity, episodic...

Homo sapiens

Synonyms: DYT9
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Disease relevance of CSE

  • In conclusion, CSE expression and H(2)S production are reduced during the development of balloon injury-induced neointimal hyperplasia, and treatment with NaHS significantly reduces neointimal lesion formation [1].
  • The other was found post-operatively to have herpes simplex virus type 1 (HSV-1) DNA and elevated titers of HSV IgM antibodies in the CSE He was given acyclovir but after initial improvement developed hemiparesis, with extensive signal change on MRI [2].
  • A decreased level of consciousness and speech disorders were more common in the LAD + CSE subgroup (P<.001) [3].
  • Hypertension, cardiac ischemia, and a history of atrial fibrillation predominated in the LAD + SAD + CSE subgroup (P<.001), while cigarette smoking was more prevalent in the LAD + SAD subgroup (P<.05) [3].
  • PURPOSE: We examined the association between seizure clustering and convulsive status epilepticus (SE) in patients with intractable complex partial seizures, to identify whether patients whose seizures typically cluster are at high risk for convulsive SE (CSE) [4].

Psychiatry related information on CSE

  • An exploratory factor analysis of the CPSS responses identified 3 factors: self-efficacy for pain management (PSE), self-efficacy for coping with symptoms (CSE), and self-efficacy for physical function (FSE) [5].
  • Furthermore, the author explored whether group identification and self-stereotyping moderated the effect of public CSE on in-group favoritism [6].
  • In 2 studies, the author examined the effect of collective self-esteem (CSE; J. Crocker & R. Luhtanen, 1993) on people's willingness to display in-group favoritism [6].
  • This study applied those measures (EEG-discriminant score; choice reaction time, CRT; continuous memory test, CMT; clinical-symptom/self-evaluation questionnaire, CSE) in a prospective experiment to determine whether our standard amount (time) of thrice weekly dialysis could be reduced without inducing some degree of clinical uraemia [7].

High impact information on CSE

  • The endogenous H(2)S donor L-cysteine also induced secretion that was diminished significantly by capsaicin desensitization, the CBS inhibitor amino-oxyacetic acid, and the CSE inhibitor propargylglycine [8].
  • CONCLUSIONS: These data establish a physiologic role for H(2)S in regulating the gastric microcirculation and identify CSE as a novel target for ASA/NSAIDs [9].
  • Glibenclamide, a K(ATP) channel inhibitor, and DL-propargylglycine, a CSE inhibitor, exacerbated, whereas pinacidil, a K(ATP) opener, attenuated gastric injury caused by ASA [9].
  • Endogenous hydrogen sulfide (H(2)S), generated from homocysteine metabolism mainly catalyzed by cystathionine gamma-lyase (CSE), possesses important functions in the cardiovascular system [1].
  • The CSE/H2S system has been shown to play an important role in regulating cellular functions in different systems [10].

Chemical compound and disease context of CSE

  • 41 of these patients had meningitis diagnosed by positive CSE culture (38) or by positive CSF latex agglutination (3); 27/41 patients also had positive blood cultures [11].
  • The objective of the present study was therefore to assess the ability of both cryptolepine (CLP) and the traditional anti-malarial formulation, the aqueous extract from the roots (CSE) to induce mutation at the hprt locus and micronuclei (MN) formation in V79, a Chinese hamster fibroblast cell line commonly used in genetic toxicity studies [12].

Biological context of CSE

  • This gene locus on chromosome 12p (HGMW-approved symbol CSE) was excluded in a large pedigree with paroxysmal choreoathetosis and additional spasticity [13].
  • In the present study, we used CSE stably overexpressed HEK-293 cells to explore the effect of the CSE/H2S system on cell growth and proliferation [10].
  • The overexpression of CSE resulted in increases in CSE mRNA levels, CSE proteins, and intracellular H2S production rates, as well as the inhibition of cell proliferation and DNA synthesis [10].
  • The gas phase of CSE did not cause apoptosis [14].
  • This study revealed that our newly developed cell seeding technique with multiple cycles of suction is a promising approach to inoculating cells into microporous sponges with high CSE, high cell viability, and homogeneous cell distribution [15].

Anatomical context of CSE

  • Moreover, NO production in LPS-stimulated macrophages that are expressing CSE mRNA was significantly reduced by the addition of L-Cys, a substrate for H(2)S, but enhanced by the selective CSE inhibitor beta-cyano-L-alanine but not by the CBS inhibitor aminooxyacetic acid [16].
  • In the present studies, monocyte procoagulants and different GAGs including chondroitin sulfate (CS) A, CSB, CSC, CSD, CSE, and heparan sulfate, were tested either in clotting assays with whole plasma or in chromogenic assays with purified coagulation proteases [17].
  • Also, isolated arteries were exposed to cigarette smoke extract (CSE; 0 to 40 mug/ml, for 6 h) in organoid culture [18].
  • Of 53 patients with temporal lobe epilepsy, CSE occurred in 13 (50%) of 26 patients with clustered seizures, and four (14.8%) of 27 patients with nonclustered seizures (p < 0.006) [4].
  • Eighteen dentin specimens were bonded to composite using CSE or SB [19].

Associations of CSE with chemical compounds


Regulatory relationships of CSE


Other interactions of CSE

  • Cystathionine gamma-lyase (CSE) is a key enzyme in the trans-sulfuration pathway, which uses L-cysteine to produce hydrogen sulfide (H2S) [23].
  • In contrast, DL-propargylglycine (CSE inhibitor, 50 mg/kg ip), exhibited marked anti-inflammatory activity as evidenced by reduced lung and liver MPO activity, and ameliorated lung and liver tissue damage [24].
  • CONCLUSIONS: CSE anesthesia is a viable anesthetic option for conventional AAA surgery in patients with severe COPD since it can preserve spontaneous breathing and provide additional respiratory benefits over general anesthesia [25].
  • CONCLUSIONS: Patients with intractable complex partial or localization-related epilepsy who typically experience seizure clustering are at a significantly higher risk for CSE than are patients with nonclustered seizures [4].
  • In cultured endothelial cells, CSE elicited NF-kappaB activation and increased monocyte adhesiveness, which were prevented by apocynin and catalase [18].

Analytical, diagnostic and therapeutic context of CSE

  • Reciprocal titration experiments with enzyme and substrate and Scatchard type analyses were consistent with concentration-dependent inhibitory interactions between CSE and sites on both factor VIIIa and IXa [17].
  • Between-day migration time precision is 1.3% for CSE and better than 0.6% for MECC [26].
  • The evaluation methodology used within the CSE project is described as a basis for presentation of results which are published elsewhere in this issue [27].
  • CONCLUSION: The results of this investigation showed a significant increase in lipid-lowering therapy with a shift from fibrates to the more innovative CSE-inhibitors in the treatment pattern of patients following hospitalization [28].
  • Asystole with successful resuscitation, occurred in 1 patient with CSE but, overall, hemodynamic perturbations were more common with the continuous techniques [29].


  1. Protective effect of hydrogen sulfide on balloon injury-induced neointima hyperplasia in rat carotid arteries. Meng, Q.H., Yang, G., Yang, W., Jiang, B., Wu, L., Wang, R. Am. J. Pathol. (2007) [Pubmed]
  2. Chronic granulomatous herpes simplex encephalitis in children. Love, S., Koch, P., Urbach, H., Dawson, T.P. J. Neuropathol. Exp. Neurol. (2004) [Pubmed]
  3. Coexisting causes of ischemic stroke. Moncayo, J., Devuyst, G., Van Melle, G., Bogousslavsky, J. Arch. Neurol. (2000) [Pubmed]
  4. The association between seizure clustering and convulsive status epilepticus in patients with intractable complex partial seizures. Haut, S.R., Shinnar, S., Moshé, S.L., O'Dell, C., Legatt, A.D. Epilepsia (1999) [Pubmed]
  5. Development and initial validation of a scale to measure self-efficacy beliefs in patients with chronic pain. Anderson, K.O., Dowds, B.N., Pelletz, R.E., Edwards, W.T., Peeters-Asdourian, C. Pain (1995) [Pubmed]
  6. Relations of self-esteem concerns, group identification, and self-stereotyping to in-group favoritism. De Cremer, D. The Journal of social psychology. (2001) [Pubmed]
  7. Assessing the adequacy of dialysis. Teschan, P.E., Ginn, H.E., Bourne, J.R., Ward, J.W. Proceedings of the European Dialysis and Transplant Association. European Dialysis and Transplant Association. (1981) [Pubmed]
  8. Hydrogen sulfide is a novel prosecretory neuromodulator in the Guinea-pig and human colon. Schicho, R., Krueger, D., Zeller, F., Von Weyhern, C.W., Frieling, T., Kimura, H., Ishii, I., De Giorgio, R., Campi, B., Schemann, M. Gastroenterology (2006) [Pubmed]
  9. Inhibition of hydrogen sulfide generation contributes to gastric injury caused by anti-inflammatory nonsteroidal drugs. Fiorucci, S., Antonelli, E., Distrutti, E., Rizzo, G., Mencarelli, A., Orlandi, S., Zanardo, R., Renga, B., Di Sante, M., Morelli, A., Cirino, G., Wallace, J.L. Gastroenterology (2005) [Pubmed]
  10. Cystathionine gamma-lyase overexpression inhibits cell proliferation via a H2S-dependent modulation of ERK1/2 phosphorylation and p21Cip/WAK-1. Yang, G., Cao, K., Wu, L., Wang, R. J. Biol. Chem. (2004) [Pubmed]
  11. Aztreonam in the treatment of gram-negative meningitis and other gram-negative infections. Feris, J., Moledina, N., Rodriguez, W.J., Khan, W.N., Puig, J., Wiedermann, B.L., Ahmad, S. Chemotherapy. (1989) [Pubmed]
  12. In vitro genotoxicity of the West African anti-malarial herbal Cryptolepis sanguinolenta and its major alkaloid cryptolepine. Ansah, C., Khan, A., Gooderham, N.J. Toxicology (2005) [Pubmed]
  13. A gene for autosomal dominant paroxysmal choreoathetosis/spasticity (CSE) maps to the vicinity of a potassium channel gene cluster on chromosome 1p, probably within 2 cM between D1S443 and D1S197. Auburger, G., Ratzlaff, T., Lunkes, A., Nelles, H.W., Leube, B., Binkofski, F., Kugel, H., Heindel, W., Seitz, R., Benecke, R., Witte, O.W., Voit, T. Genomics (1996) [Pubmed]
  14. Acute cigarette smoke exposure induces apoptosis of alveolar macrophages. Aoshiba, K., Tamaoki, J., Nagai, A. Am. J. Physiol. Lung Cell Mol. Physiol. (2001) [Pubmed]
  15. New technique of seeding chondrocytes into microporous poly(L-lactide-co-epsilon-caprolactone) sponge by cyclic compression force-induced suction. Xie, J., Jung, Y., Kim, S.H., Kim, Y.H., Matsuda, T. Tissue Eng. (2006) [Pubmed]
  16. Hydrogen sulfide inhibits nitric oxide production and nuclear factor-kappaB via heme oxygenase-1 expression in RAW264.7 macrophages stimulated with lipopolysaccharide. Oh, G.S., Pae, H.O., Lee, B.S., Kim, B.N., Kim, J.M., Kim, H.R., Jeon, S.B., Jeon, W.K., Chae, H.J., Chung, H.T. Free Radic. Biol. Med. (2006) [Pubmed]
  17. Specific regulation of procoagulant activity on monocytes. Intrinsic pathway inhibition by chondroitin 4,6-disulfate. McGee, M.P., Teuschler, H., Parthasarathy, N., Wagner, W.D. J. Biol. Chem. (1995) [Pubmed]
  18. Cigarette smoke-induced proinflammatory alterations in the endothelial phenotype: role of NAD(P)H oxidase activation. Orosz, Z., Csiszar, A., Labinskyy, N., Smith, K., Kaminski, P.M., Ferdinandy, P., Wolin, M.S., Rivera, A., Ungvari, Z. Am. J. Physiol. Heart Circ. Physiol. (2007) [Pubmed]
  19. Effect of hydration variability on hybrid layer properties of a self-etching versus an acid-etching system. Schulze, K.A., Oliveira, S.A., Wilson, R.S., Gansky, S.A., Marshall, G.W., Marshall, S.J. Biomaterials (2005) [Pubmed]
  20. Characterization of a neutrophil cell surface glycosaminoglycan that mediates binding of platelet factor 4. Petersen, F., Brandt, E., Lindahl, U., Spillmann, D. J. Biol. Chem. (1999) [Pubmed]
  21. Parallel variation of ventricular CSF tryptophan and free serum tryptophan in man. Young, S.N., Lal, S., Feldmuller, F., Sourkes, T.L., Ford, R.M., Kiely, M., Martin, J.B. J. Neurol. Neurosurg. Psychiatr. (1976) [Pubmed]
  22. Extracts from cigarette smoke induce DNA damage and cell adhesion molecule expression through different pathways. Chen, H.W., Chien, M.L., Chaung, Y.H., Lii, C.K., Wang, T.S. Chem. Biol. Interact. (2004) [Pubmed]
  23. Pro-apoptotic effect of endogenous H2S on human aorta smooth muscle cells. Yang, G., Wu, L., Wang, R. FASEB J. (2006) [Pubmed]
  24. Hydrogen sulfide is a novel mediator of lipopolysaccharide-induced inflammation in the mouse. Li, L., Bhatia, M., Zhu, Y.Z., Zhu, Y.C., Ramnath, R.D., Wang, Z.J., Anuar, F.B., Whiteman, M., Salto-Tellez, M., Moore, P.K. FASEB J. (2005) [Pubmed]
  25. Combined spinal and epidural anesthesia for abdominal aortic aneurysm surgery in patients with severe chronic pulmonary obstructive disease. Flores, J.A., Nishibe, T., Koyama, M., Imai, T., Kudo, F., Miyazaki, K., Yasuda, K. International angiology : a journal of the International Union of Angiology. (2002) [Pubmed]
  26. Reproducible two-dimensional capillary electrophoresis analysis of Barrett's esophagus tissues. Kraly, J.R., Jones, M.R., Gomez, D.G., Dickerson, J.A., Harwood, M.M., Eggertson, M., Paulson, T.G., Sanchez, C.A., Odze, R., Feng, Z., Reid, B.J., Dovichi, N.J. Anal. Chem. (2006) [Pubmed]
  27. Reference standards for software evaluation. Michaelis, J., Wellek, S., Willems, J.L. Methods of information in medicine. (1990) [Pubmed]
  28. Lipid-lowering therapy: do hospitals influence the prescribing behavior of general practitioners? Schröder-Bernhardi, D., Dietlein, G. International journal of clinical pharmacology and therapeutics. (2002) [Pubmed]
  29. Technical aspects and postoperative sequelae of spinal and epidural anesthesia: a prospective study of 3,230 orthopedic patients. Puolakka, R., Haasio, J., Pitkänen, M.T., Kallio, M., Rosenberg, P.H. Regional anesthesia and pain medicine. (2000) [Pubmed]
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