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Gene Review

PLOD2  -  procollagen-lysine, 2-oxoglutarate 5...

Homo sapiens

Synonyms: LH2, Lysyl hydroxylase 2, Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2, TLH
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Disease relevance of PLOD2

  • Together our data identify LH2 (long) as a bifunctional protein and underscores its potential role in the pathogenesis of fibrosis [1].
  • Subsequently, we investigated fibroblasts derived from fibrotic skin of systemic sclerosis (SSc) patients and found that PLOD2 mRNA is highly increased indeed [2].
  • Phenotypic and molecular characterization of Bruck syndrome (osteogenesis imperfecta with contractures of the large joints) caused by a recessive mutation in PLOD2 [3].
  • We demonstrate, by modeling the structure using the highly homologous structure of LH2 from Rhodospirillum molischianum, that it has the minimal size for BChl binding [4].
  • In this work we present and discuss the single-molecule fluorescence spectra of a variety of species of light-harvesting complexes: LH2 of Rhodopseudomonas acidophila, Rhodobacter sphaeroides, and Rhodospirillum molischianum and LH1 of Rhodobacter sphaeroides [5].

High impact information on PLOD2


Chemical compound and disease context of PLOD2


Biological context of PLOD2


Anatomical context of PLOD2

  • The increase of collagen synthesis in fibroblasts overexpressing LH2 (long) was independent from cross-linking as it was also observed in the presence of beta-aminopropionitril, a cross-linking inhibitor [1].
  • In humans, mutations in the genes encoding LH1 and LH2 have been shown to cause two distinct connective tissue disorders, Ehlers-Danlos (Type VIA) and Bruck syndromes [10].
  • The thick limb of the loop of Henle (TLH) isolated from the MT nephron of the quail Coturnix coturnix revealed a lumen-positive, furosemide-sensitive transepithelial voltage (Vt) that requires the presence of both Na and Cl [11].
  • There were 263 operations of the benign adnexal masses, and 107 operations due to benign tumors of corpus uteri (there were 18 LAVH, 12 LH and 77 TLH) [12].

Associations of PLOD2 with chemical compounds

  • His urine contained high amounts of hydroxyproline but low amounts of collagen crosslinks degradation products; and he was shown to be homozygous for a novel mutation leading to an Arg598His substitution in PLOD2 [3].
  • The increase in pyridinoline cross-links is likely to be the result of increased activity of the enzyme responsible for the hydroxylation of the telopeptides (telopeptide lysyl hydroxylase, or TLH) [2].
  • Lysyl hydroxylase 2 contains nine cysteine residues, which are conserved in lysyl hydroxylase 1 [13].
  • In kidney cells, in which both LH2 transcripts are equally expressed, the long LH2 transcript is significantly decreased after 24 h CHX treatment, whereas expression of the short transcript is slightly increased [14].
  • Regulation of biosynthesis and assembly 2574.1 Regulation 2574.1.1 Oxygen 2574.1.2 Light 2584.1.2.1 AppA: blue-light-mediated regulation 2594.1.2.2 Bacteriophytochromes 2594.1.3 From the RC to the mature PSU 2614.2 Assembly 2614.2.1 LH1 2624.2.2 LH2 2635 [15].

Physical interactions of PLOD2

  • Structural analysis of crystallized peripheral (LH2) and core antenna complexes (LH1) of purple bacteria has revealed circular aggregates of high rotational symmetry (C8, C9 and C16, respectively) [16].

Regulatory relationships of PLOD2


Analytical, diagnostic and therapeutic context of PLOD2

  • Amplification of cDNAs by PCR, using primers from exons 13 and 14, showed the presence of two distinct LH2 mRNA populations [18].
  • The steady-state absorption and fluorescence spectroscopy of the B808-866 complex are reminiscent of the well-studied LH2 peripheral antenna of purple bacteria, whereas the protein sequence and the circular dichroism spectrum of B808-866 is more similar to the LH1 inner core antenna [19].
  • In this work we have investigated the carotenoid-protein interactions in LH2 complexes of Rhodopseudomonas acidophila both in "free in solution" mixed-micelles and in three-dimensional crystals by Raman spectroscopy in resonance with the carotenoid (Car) molecules [20].
  • Of these 88 patients, 61 had TLH while 27 patients had indicated pelvic and aortic node dissection [21].
  • OBJECTIVE.: We sought to analyze surgical results of women with uterine cancers having TLH +/- staging, stratifying data by body mass index (BMI) [21].


  1. Functional diversity of lysyl hydroxylase 2 in collagen synthesis of human dermal fibroblasts. Wu, J., Reinhardt, D.P., Batmunkh, C., Lindenmaier, W., Far, R.K., Notbohm, H., Hunzelmann, N., Brinckmann, J. Exp. Cell Res. (2006) [Pubmed]
  2. Identification of PLOD2 as telopeptide lysyl hydroxylase, an important enzyme in fibrosis. van der Slot, A.J., Zuurmond, A.M., Bardoel, A.F., Wijmenga, C., Pruijs, H.E., Sillence, D.O., Brinckmann, J., Abraham, D.J., Black, C.M., Verzijl, N., DeGroot, J., Hanemaaijer, R., TeKoppele, J.M., Huizinga, T.W., Bank, R.A. J. Biol. Chem. (2003) [Pubmed]
  3. Phenotypic and molecular characterization of Bruck syndrome (osteogenesis imperfecta with contractures of the large joints) caused by a recessive mutation in PLOD2. Ha-Vinh, R., Alanay, Y., Bank, R.A., Campos-Xavier, A.B., Zankl, A., Superti-Furga, A., Bonafé, L. Am. J. Med. Genet. A (2004) [Pubmed]
  4. Design of a minimal polypeptide unit for bacteriochlorophyll binding and self-assembly based on photosynthetic bacterial light-harvesting proteins. Noy, D., Dutton, P.L. Biochemistry (2006) [Pubmed]
  5. Spectral trends in the fluorescence of single bacterial light-harvesting complexes: experiments and modified redfield simulations. Rutkauskas, D., Novoderezhkin, V., Gall, A., Olsen, J., Cogdell, R.J., Hunter, C.N., van Grondelle, R. Biophys. J. (2006) [Pubmed]
  6. Differential expression of human lysyl hydroxylase genes, lysine hydroxylation, and cross-linking of type I collagen during osteoblastic differentiation in vitro. Uzawa, K., Grzesik, W.J., Nishiura, T., Kuznetsov, S.A., Robey, P.G., Brenner, D.A., Yamauchi, M. J. Bone Miner. Res. (1999) [Pubmed]
  7. Preferential incorporation of coloured-carotenoids occurs in the LH2 complexes from non-sulphur purple bacteria under carotenoid-limiting conditions. Gall, A., Henry, S., Takaichi, S., Robert, B., Cogdell, R.J. Photosyn. Res. (2005) [Pubmed]
  8. Complete genomic structure of mouse lysyl hydroxylase 2 and lysyl hydroxylase 3/collagen glucosyltransferase. Ruotsalainen, H., Vanhatupa, S., Tampio, M., Sipilä, L., Valtavaara, M., Myllylä, R. Matrix Biol. (2001) [Pubmed]
  9. Decreased expression of lysyl hydroxylase 2 (LH2) in skin fibroblasts from three Ehlers-Danlos patients does not result from mutations in either the coding or proximal promoter region of the LH2 gene. Walker, L.C., Teebi, A.S., Marini, J.C., De Paepe, A., Malfait, F., Atsawasuwan, P., Yamauchi, M., Yeowell, H.N. Mol. Genet. Metab. (2004) [Pubmed]
  10. Genomic structure and embryonic expression of zebrafish lysyl hydroxylase 1 and lysyl hydroxylase 2. Schneider, V.A., Granato, M. Matrix Biol. (2007) [Pubmed]
  11. Renal handling of sodium chloride and its control in birds. Nishimura, H., Miwa, T., Bailey, J.R. J. Exp. Zool. (1984) [Pubmed]
  12. Minimally invasive surgery in the field of gynecologic oncology. Kopjar, M., Buković, D., Zadro, M., Fures, R., Maricić, I. Collegium antropologicum. (1999) [Pubmed]
  13. Cloning and characterization of a novel human lysyl hydroxylase isoform highly expressed in pancreas and muscle. Valtavaara, M., Papponen, H., Pirttilä, A.M., Hiltunen, K., Helander, H., Myllylä, R. J. Biol. Chem. (1997) [Pubmed]
  14. Tissue-specific expression and regulation of the alternatively-spliced forms of lysyl hydroxylase 2 (LH2) in human kidney cells and skin fibroblasts. Walker, L.C., Overstreet, M.A., Yeowell, H.N. Matrix Biol. (2005) [Pubmed]
  15. The architecture and function of the light-harvesting apparatus of purple bacteria: from single molecules to in vivo membranes. Cogdell, R.J., Gall, A., K??hler, J. Q. Rev. Biophys. (2006) [Pubmed]
  16. Pigment-protein architecture in the light-harvesting antenna complexes of purple bacteria: does the crystal structure reflect the native pigment-protein arrangement? Leupold, D., Voigt, B., Beenken, W., Stiel, H. FEBS Lett. (2000) [Pubmed]
  17. Interleukin 4 and prolonged hypoxia induce a higher gene expression of lysyl hydroxylase 2 and an altered cross-link pattern: important pathogenetic steps in early and late stage of systemic scleroderma? Brinckmann, J., Kim, S., Wu, J., Reinhardt, D.P., Batmunkh, C., Metzen, E., Notbohm, H., Bank, R.A., Krieg, T., Hunzelmann, N. Matrix Biol. (2005) [Pubmed]
  18. Tissue specificity of a new splice form of the human lysyl hydroxylase 2 gene. Yeowell, H.N., Walker, L.C. Matrix Biol. (1999) [Pubmed]
  19. Purification and characterization of the B808-866 light-harvesting complex from green filamentous bacterium Chloroflexus aurantiacus. Xin, Y., Lin, S., Montaño, G.A., Blankenship, R.E. Photosyn. Res. (2005) [Pubmed]
  20. Carotenoid stoichiometry in the LH2 crystal: no spectral evidence for the presence of the second molecule in the alpha/beta-apoprotein dimer. Gall, A., Gardiner, A.T., Cogdell, R.J., Robert, B. FEBS Lett. (2006) [Pubmed]
  21. Total laparoscopic hysterectomy for uterine pathology: Impact of body mass index on outcomes. O'hanlan, K.A., Dibble, S.L., Fisher, D.T. Gynecol. Oncol. (2006) [Pubmed]
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