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Gene Review

LCS1  -  lymphedema-cholestasis syndrome 1

Homo sapiens

Synonyms: CHLS, CHSL, LCS
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Disease relevance of LCS1


Psychiatry related information on LCS1


High impact information on LCS1

  • Modeling using the x-ray structure of cyanobacterial PSII suggests that energy transfer to the PSII reaction center is via the Chls bound to the CP47 and CP43 proteins [5].
  • From HPLC analysis, the pigment stoichiometry was suggested to be 5 Chls per 1 beta-carotene per 2 pheophytins [6].
  • WSCP is the first example of a hydrophilic protein that can transfer Chls from thylakoid hydrophobic proteins [7].
  • When the recombinant MBP-WSCP was incubated with thylakoid membranes, the MBP-WSCP removed Chls from these membranes [7].
  • GnRH (gonadotropin releasing hormone) augmented the size of both pools to a larger extent (5- and 1.7-fold increase for HCSP and LCSP, respectively) [8].

Biological context of LCS1


Anatomical context of LCS1

  • Further experiments were carried out to verify that phytanic acid was activated by LCS in rat liver peroxisomes [11].
  • A 63-year-old man with osteoarthritis of the right knee underwent TKA with a New Jersey LCS Knee, with cementing on the tibia and patella but not on the femoral component [12].
  • The posterior cruciate ligament (PCL)-retaining, meniscal bearing and the PCL-sacrificing rotating platform designs of the LCS prosthesis (DePuy, Warsaw, IN) were designed to minimally constrain knee kinematics while minimizing bone-cement-prosthesis interface stresses and polyethylene wear [13].
  • This study presents a synopsis of the senior author's (LCS) experience with 439 surgically treated patients with hallux metatarsophalangeal arthritis, focusing on origin and treatment [14].
  • Laser scanning cytometry (LCS) allows detailed analysis of the cell cycle in PI stained human fibroblasts (TIG-7) [15].

Associations of LCS1 with chemical compounds

  • The LCS1 sequence is present in all Luzula species tested so far (except L. pilosa) and like other satellite repeats found in heterochromatin, the cytosine residues are methylated within the LCS1 repeats [9].
  • Attempts to separate LCS from phytanoyl-CoA synthetase by chromatography on several matrices were unsuccessful [11].
  • To test directly whether LCS could activate phytanic acid, rat liver cDNA encoding this enzyme was transcribed and translated in vitro [11].
  • This decay is proposed to be related to the presence of the interacting Chls A5 and B5 located next to the carotenoid in the site L2 and may imply some photoprotective role for Lhca4 in the photosystem I super-complex [16].
  • The expressed enzyme had both LCS activity (assayed with palmitic acid, C16: 0) and phytanoyl-CoA synthetase activity; VLCS activity (assayed with lignoceric acid, C24: 0) was not detectable [11].

Analytical, diagnostic and therapeutic context of LCS1

  • Using fluorescent in situ hybridization (FISH) experiments we have shown that there are at least 5 large clusters of LCS1 sequences distributed at heterochromatin regions along each of the 12 chromosomes of L. nivea [9].
  • Preparative isoelectric focusing revealed that phytanoyl-CoA synthetase and LCS had indistinguishable isoelectric points [11].
  • METHODS: Between November 1985 and November 1988, the senior author (R. C. J.) performed 119 consecutive total knee arthroplasties in eighty-six patients with LCS rotating-platform femoral and tibial components and a Townley all-polyethylene dome patellar component [17].
  • The LCS (low contact stress) prosthesis was used initially, in nineteen ankles, between 1988 and 1992, and a modification of the LCS prosthesis, the Buechel-Pappas design, was used in seventy-four ankles between 1993 and 1999 [18].
  • Clinical and radiographic analyses and survivorship were done on the initial cementless series of 309 posterior cruciate-retaining meniscal-bearing and rotating-platform New Jersey LCS total knee replacements followed up for 10 to 20 years (mean, 12.4 years) [19].


  1. Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome). Drivdal, M., Trydal, T., Hagve, T.A., Bergstad, I., Aagenaes, O. Scand. J. Gastroenterol. (2006) [Pubmed]
  2. Evidence for genetic heterogeneity in lymphedema-cholestasis syndrome. Frühwirth, M., Janecke, A.R., Müller, T., Carlton, V.E., Kronenberg, F., Offner, F., Knisely, A.S., Geleff, S., Song, E.J., Simma, B., Königsrainer, A., Margreiter, R., van der Hagen, C.B., Eiklid, K., Aagenaes, O., Bull, L., Ellemunter, H. J. Pediatr. (2003) [Pubmed]
  3. Echocardiographic diagnosis of aneurysm of the sinus of Valsalva. Dev, V., Goswami, K.C., Shrivastava, S., Bahl, V.K., Saxena, A. Am. Heart J. (1993) [Pubmed]
  4. CHSLP pastoral care model. Wheelock, R.E. Hospital progress. (1975) [Pubmed]
  5. Structure of a photosystem II supercomplex isolated from Prochloron didemni retaining its chlorophyll a/b light-harvesting system. Bibby, T.S., Nield, J., Chen, M., Larkum, A.W., Barber, J. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  6. Photochemistry and spectroscopy of a five-chlorophyll reaction center of photosystem II isolated by using a Cu affinity column. Vacha, F., Joseph, D.M., Durrant, J.R., Telfer, A., Klug, D.R., Barber, J. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  7. Molecular cloning and functional expression of a water-soluble chlorophyll protein, a putative carrier of chlorophyll molecules in cauliflower. Satoh, H., Nakayama, K., Okada, M. J. Biol. Chem. (1998) [Pubmed]
  8. Heterogeneity of the Ca(2+) sensitivity of secretion in a pituitary gonadotrope cell line and its modulation by protein kinase C and Ca(2+). Liu, H.S., Hu, Z.T., Zhou, K.M., Jiu, Y.M., Yang, H., Wu, Z.X., Xu, T. J. Cell. Physiol. (2006) [Pubmed]
  9. Molecular analysis of holocentric centromeres of Luzula species. Haizel, T., Lim, Y.K., Leitch, A.R., Moore, G. Cytogenet. Genome Res. (2005) [Pubmed]
  10. Assignment of spectral substructures to pigment-binding sites in higher plant light-harvesting complex LHC-II. Rogl, H., Schödel, R., Lokstein, H., Kühlbrandt, W., Schubert, A. Biochemistry (2002) [Pubmed]
  11. Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase. Watkins, P.A., Howard, A.E., Gould, S.J., Avigan, J., Mihalik, S.J. J. Lipid Res. (1996) [Pubmed]
  12. Fracture of the femoral component associated with polyethylene wear and osteolysis after total knee arthroplasty. Huang, C.H., Yang, C.Y., Cheng, C.K. The Journal of arthroplasty. (1999) [Pubmed]
  13. Kinematics of posterior cruciate ligament-retaining and -sacrificing mobile bearing total knee arthroplasties. An in vitro comparison of the New Jersey LCS meniscal bearing and rotating platform prostheses. Lewandowski, P.J., Askew, M.J., Lin, D.F., Hurst, F.W., Melby, A. The Journal of arthroplasty. (1997) [Pubmed]
  14. Hallux metatarsophalangeal arthritis. Weinfeld, S.B., Schon, L.C. Clin. Orthop. Relat. Res. (1998) [Pubmed]
  15. Laser scanning cytometry (LCS) allows detailed analysis of the cell cycle in PI stained human fibroblasts (TIG-7). Kawasaki, M., Sasaki, K., Satoh, T., Kurose, A., Kamada, T., Furuya, T., Murakami, T., Todoroki, T. Cell Prolif. (1997) [Pubmed]
  16. Excitation energy transfer pathways in Lhca4. Gibasiewicz, K., Croce, R., Morosinotto, T., Ihalainen, J.A., van Stokkum, I.H., Dekker, J.P., Bassi, R., van Grondelle, R. Biophys. J. (2005) [Pubmed]
  17. Cemented rotating-platform total knee replacement. A nine to twelve-year follow-up study. Callaghan, J.J., Squire, M.W., Goetz, D.D., Sullivan, P.M., Johnston, R.C. The Journal of bone and joint surgery. American volume. (2000) [Pubmed]
  18. Total ankle arthroplasty in inflammatory joint disease with use of two mobile-bearing designs. Doets, H.C., Brand, R., Nelissen, R.G. The Journal of bone and joint surgery. American volume. (2006) [Pubmed]
  19. Long-term followup after mobile-bearing total knee replacement. Buechel, F.F. Clin. Orthop. Relat. Res. (2002) [Pubmed]
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