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Gene Review

CSH2  -  chorionic somatomammotropin hormone 2

Homo sapiens

Synonyms: CS-2, CSB, PL, hCS-B
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Disease relevance of CSH2


Psychiatry related information on CSH2

  • Only the group exposed to values exceeding three times the recommended TLV for CS2; had significant impairments in both the speed and the quality of psychomotor performance [6].

High impact information on CSH2

  • In the shocked jovian atmosphere, HCS could further decompose to H and CS, and CS could react with SH and OH to yield the observed CS2 and COS [7].
  • Mutations in the CSA or CSB complementation genes cause the Cockayne syndrome, a severe genetic disorder that results in patients' death in early adulthood [2].
  • From these studies we concluded that normal and atherosclerotic human aortas contain CSB, CSC, HA, and HS [8].
  • Further, we demonstrated that CSB is a hybrid consisting of approximately 40% CSA and 60% CSB and that CSC appears to be a polymer consisting essentially of glucuronic acid and N-acetylgalactosamine-6-sulfate [8].
  • Remarkably, many of the genes regulated by CSB are also affected by inhibitors of histone deacetylase and DNA methylation, as well as by defects in poly(ADP-ribose)-polymerase function and RNA polymerase II elongation [9].

Chemical compound and disease context of CSH2


Biological context of CSH2

  • The retention of only the hCS-B gene was associated with normal weight and length at birth and normal postpartum lactation in the mother heterozygous for the deletion [14].
  • The end points of the deletion lay within two regions of highly homologous DNA sequence situated 5' to the hGH-1 gene and 5' to the hCS-B gene [14].
  • We also studied the regions of the hCS-L and hCS-A genes which are highly similar to the hCS-B enhancer [15].
  • Starting from approximately equal levels at 8 weeks of gestation, hCS-A is expressed 5-fold more abundantly than hCS-B by term [16].
  • In addition, the CSB mutant alleles were tested for their ability to complement the sensitivity of UV61 cells to the carcinogen 4-nitroquinoline-1-oxide (4-NQO), which introduces bulky DNA adducts repaired by global genome repair [17].

Anatomical context of CSH2

  • The human placental lactogen B (hCS-B) promoter activity is strongly stimulated by thyroid hormones in the rat pituitary GC cell line [18].
  • The human CSB (ERCC6) gene corrects the transcription-coupled repair defect in the CHO cell mutant UV61 [19].
  • 8-oxoG repair was measured in liver cells from CSB deficient mice and in human CS-B cells carrying expression vectors for wild type or mutant forms of the human CSB gene [3].
  • Two commonly employed solvents, n-hexane and carbon disulfide (CS2), although chemically dissimilar, result in identical neurofilament-filled swellings of the distal axon in both the central and peripheral nervous systems [20].
  • Increased NBT reductions by PMN's was found when leukocytes were incubated with heparin, CSA, and CSB, but these compounds had no effect on the HMPS activity of PMN's unless NBT was added [21].

Associations of CSH2 with chemical compounds

  • Point mutation of a highly conserved glutamic acid residue in ATPase motif II abolished the ability of CSB protein to complement the UV-sensitive phenotypes of survival, RNA synthesis recovery, and gene-specific repair [17].
  • The International Ultraviolet Explorer and HST spectra showed emissions from OH (a tracer of H2O) and CS (a tracer of CS2) starting in April 1996, and from the CO Cameron system (which primarily traces CO2) starting in June 1996 [22].
  • A purification procedure for C60 from the fullerene extract has been developed using the differential complexation of C60 and C70 with AlCl3 (or its conjugate acid) in CS2 solution [23].
  • Human placental lactogen B (hCS-B) promoter activity is strongly stimulated by triiodothyronine (T3) in pituitary GC cells through interaction between the thyroid receptor and a thyroid receptor-binding element (TBE) spanning coordinates -67 to -41 [24].
  • Both HD and CS2 react with protein amino functions to yield initial adducts (pyrrolyl or dithiocarbamate derivatives, respectively), which then undergo oxidation or decomposition to an electrophile (oxidized pyrrole ring or isothiocyanate), that then reacts with protein nucleophiles to result in protein cross-linking [20].

Physical interactions of CSH2

  • A TEF-1 binding motif that interacts with a placental protein is important for the transcriptional activity of the hCS-B enhancer [25].

Regulatory relationships of CSH2

  • These results strongly suggest that hTEF-5 regulates the activity of the hCS-B gene enhancer [26].
  • Furthermore, evidence is presented to support the hypothesis that wild type CSB regulates expression of OGG1 [3].

Other interactions of CSH2

  • Levels of hCS-A, hCS-B, and hGH-V transcripts are all elevated [27].
  • These data indicate that the integrity of the ATPase domain is critical for CSB function in vivo [17].
  • Traditionally, CSA and CSB have been ascribed a function in the transcription-coupled repair (TCR) pathway of nucleotide excision repair (NER) that efficiently removes bulky lesions from the transcribed strand of RNA polymerase II transcribed genes [28].
  • A "new" HLA-C specificity, CSH-2, restricted to Oriental populations [29].

Analytical, diagnostic and therapeutic context of CSH2

  • In this study, single amino acid changes in highly conserved residues of helicase motifs Ia, III, V, VI and a second putative nucleotide-binding motif (NTB) of the CSB protein were generated by site-directed mutagenesis to analyze the genetic function of the CSB protein in survival, RNA synthesis recovery and apoptosis after UV treatment [30].
  • Breakdown of CSA, CSB, and CSC was assessed by gel filtration chromatography and compared with untreated controls [31].
  • In the diagnosis of ABPA, skin tests with CS2 were comparable in specificity to currently available commercial preparations [32].
  • Analysis revealed that CS2 exposure produced significant alterations in copulatory behavior and a decrease in ejaculated sperm counts by the fourth and seventh weeks of exposure, respectively [33].
  • Antibody titres of the IgG and IgA classes to CS2, as measured by enzyme-linked immunosorbent assay (ELISA), were demonstrated to be similar in aspergilloma and ABPA patients; IgG titres were higher than IgA [32].


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  2. CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome. Groisman, R., Kuraoka, I., Chevallier, O., Gaye, N., Magnaldo, T., Tanaka, K., Kisselev, A.F., Harel-Bellan, A., Nakatani, Y. Genes Dev. (2006) [Pubmed]
  3. Mitochondrial repair of 8-oxoguanine is deficient in Cockayne syndrome group B. Stevnsner, T., Nyaga, S., de Souza-Pinto, N.C., van der Horst, G.T., Gorgels, T.G., Hogue, B.A., Thorslund, T., Bohr, V.A. Oncogene (2002) [Pubmed]
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  5. CD23 and CD21 function as adhesion molecules in homotypic aggregation of human B lymphocytes. Björck, P., Elenström-Magnusson, C., Rosén, A., Severinson, E., Paulie, S. Eur. J. Immunol. (1993) [Pubmed]
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  7. The formation of HCS and HCSH molecules and their role in the collision of comet Shoemaker-Levy 9 with Jupiter. Kaiser, R.I., Ochsenfeld, C., Head-Gordon, M., Lee, Y.T. Science (1998) [Pubmed]
  8. The glycosaminoglycans of the human artery and their changes in atherosclerosis. Stevens, R.L., Colombo, M., Gonzales, J.J., Hollander, W., Schmid, K. J. Clin. Invest. (1976) [Pubmed]
  9. Cockayne syndrome group B protein (CSB) plays a general role in chromatin maintenance and remodeling. Newman, J.C., Bailey, A.D., Weiner, A.M. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  10. Nosocomial spread of ceftazidime-resistant Klebsiella pneumoniae strains producing a novel class a beta-lactamase, GES-3, in a neonatal intensive care unit in Japan. Wachino, J., Doi, Y., Yamane, K., Shibata, N., Yagi, T., Kubota, T., Ito, H., Arakawa, Y. Antimicrob. Agents Chemother. (2004) [Pubmed]
  11. Physiology and taxonomy of thiobacillus strain TJ330, which oxidizes carbon disulphide (CS2). Hartikainen, T., Ruuskanen, J., Räty, K., Von Wright, A., Martikainen, P.J. J. Appl. Microbiol. (2000) [Pubmed]
  12. An epidemiological study on carbon disulfide angiopathy in a Chinese viscose rayon factory. Sugimoto, K., Seki, Y., Goto, S., Karai, I., Liang, Y.X., Lu, P.K., Ding, X.J., Li, M.Q., Gu, X.Q. International archives of occupational and environmental health. (1984) [Pubmed]
  13. A case comparison study of carbon disulfide retinopathy and diabetic retinopathy using fluorescein fundus angiography. Karai, I., Sugimoto, K., Goto, S. Acta ophthalmologica. (1983) [Pubmed]
  14. Isolated growth hormone (GH) deficiency type IA associated with a 45-kilobase gene deletion within the human GH gene cluster. Akinci, A., Kanaka, C., Eblé, A., Akar, N., Vidinlisan, S., Mullis, P.E. J. Clin. Endocrinol. Metab. (1992) [Pubmed]
  15. Characterization of a single strong tissue-specific enhancer downstream from the three human genes encoding placental lactogen. Jacquemin, P., Oury, C., Peers, B., Morin, A., Belayew, A., Martial, J.A. Mol. Cell. Biol. (1994) [Pubmed]
  16. Developmental control and alternative splicing of the placentally expressed transcripts from the human growth hormone gene cluster. MacLeod, J.N., Lee, A.K., Liebhaber, S.A., Cooke, N.E. J. Biol. Chem. (1992) [Pubmed]
  17. The ATPase domain but not the acidic region of Cockayne syndrome group B gene product is essential for DNA repair. Brosh, R.M., Balajee, A.S., Selzer, R.R., Sunesen, M., Proietti De Santis, L., Bohr, V.A. Mol. Biol. Cell (1999) [Pubmed]
  18. Characterization of an unusual thyroid response unit in the promoter of the human placental lactogen gene. Voz, M.L., Peers, B., Belayew, A., Martial, J.A. J. Biol. Chem. (1991) [Pubmed]
  19. The human CSB (ERCC6) gene corrects the transcription-coupled repair defect in the CHO cell mutant UV61. Orren, D.K., Dianov, G.L., Bohr, V.A. Nucleic Acids Res. (1996) [Pubmed]
  20. Pathogenetic studies of hexane and carbon disulfide neurotoxicity. Graham, D.G., Amarnath, V., Valentine, W.M., Pyle, S.J., Anthony, D.C. Crit. Rev. Toxicol. (1995) [Pubmed]
  21. The effects of polyanions on NBT Reductions hexose monophosphate shunt activity, and ultrastructure of polymorphonuclear leukocytes. Czarnetzki, B.M., Cowan, D.H., Belcher, R.W. Am. J. Clin. Pathol. (1975) [Pubmed]
  22. The activity and size of the nucleus of comet Hale-Bopp (C/1995 O1) [see comment]. Weaver, H.A., Feldman, P.D., A'Hearn, M.F., Arpigny, C., Brandt, J.C., Festou, M.C., Haken, M., McPhate, J.B., Stern, S.A., Tozzi, G.P. Science (1997) [Pubmed]
  23. Convenient separation of high-purity C60 from crude fullerene extract by selective complexation with AlCl3. Bucsi, I., Aniszfeld, R., Shamma, T., Prakash, G.K., Olah, G.A. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  24. Transcriptional regulation by triiodothyronine requires synergistic action of the thyroid receptor with another trans-acting factor. Voz, M.L., Peers, B., Wiedig, M.J., Jacquemin, P., Belayew, A., Martial, J.A. Mol. Cell. Biol. (1992) [Pubmed]
  25. A TEF-1 binding motif that interacts with a placental protein is important for the transcriptional activity of the hCS-B enhancer. Jacquemin, P., Oury, C., Belayew, A., Martial, J.A. DNA Cell Biol. (1994) [Pubmed]
  26. Human TEF-5 is preferentially expressed in placenta and binds to multiple functional elements of the human chorionic somatomammotropin-B gene enhancer. Jacquemin, P., Martial, J.A., Davidson, I. J. Biol. Chem. (1997) [Pubmed]
  27. Tissue-specific expression and thyroid hormone regulation of the endogenous placental growth hormone variant and chorionic somatomammotropin genes in a human choriocarcinoma cell line. Nickel, B.E., Cattini, P.A. Endocrinology (1991) [Pubmed]
  28. Global genome repair of 8-oxoG in hamster cells requires a functional CSB gene product. Sunesen, M., Stevnsner, T., Brosh Jr, R.M., Dianov, G.L., Bohr, V.A. Oncogene (2002) [Pubmed]
  29. A "new" HLA-C specificity, CSH-2, restricted to Oriental populations. Zhao, T.M., Lee, T.D., Bu, K.J., Zhang, G.L., Zheng, S.Q., Gu, W.J., Lee, P.Y., Rubinstein, P. Tissue Antigens (1987) [Pubmed]
  30. Phenotypic consequences of mutations in the conserved motifs of the putative helicase domain of the human Cockayne syndrome group B gene. Muftuoglu, M., Selzer, R., Tuo, J., Brosh, R.M., Bohr, V.A. Gene (2002) [Pubmed]
  31. Nitric oxide products degrade chondroitin sulfates. Hassan, M.S., Mileva, M.M., Dweck, H.S., Rosenfeld, L. Nitric Oxide (1998) [Pubmed]
  32. Antigens of Aspergillus fumigatus. II. Electrophoretic and clinical studies. Piechura, J.E., Huang, C.J., Cohen, S.H., Kidd, J.M., Kurup, V.P., Calvanico, N.J. Immunology (1983) [Pubmed]
  33. An evaluation of the copulatory, endocrinologic, and spermatotoxic effects of carbon disulfide in the rat. Zenick, H., Blackburn, K., Hope, E., Baldwin, D. Toxicol. Appl. Pharmacol. (1984) [Pubmed]
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