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Gene Review

Papss2  -  3'-phosphoadenosine 5'-phosphosulfate...

Mus musculus

Synonyms: 1810018P12Rik, AI159688, AtpsU2, Atpsk2, Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2, ...
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Disease relevance of Papss2

  • We investigated whether loss of Papss2 activity would similarly lead to degenerative joint disease in mice [1].
  • These results suggest that both bm/bm and A/J exhibit a delay in palatal shelf rotation and elevated levels of cyclic AMP, which appear to be predisposing factors for cortisone-induced cleft palate [2].
  • Here, we have addressed the role of MHC class I bound peptides in NK cell mediated F1-hybrid anti-parental rejection by studying the resistance of F1-hybrids between B6 and different bm mutant strains to B6-derived RBL-5 lymphoma cell line [3].

High impact information on Papss2

  • We characterized a nonsense mutation in ATPSK2 in the SEMD family and a missense mutation in the region of Atpsk2 encoding the APS kinase activity in the brachymorphic mouse [4].
  • Gene conversion between murine class II major histocompatibility complex loci. Functional and molecular evidence from the bm 12 mutant [5].
  • Newborn bm/bm epiphyseal cartilages are shorter than normal although the cells in the different zones of growth are relatively well organized [6].
  • In addition, a prominent network of fine filaments, which are extractable in 4 M guanidine hydrochloride, are present in the bm/bm cartilage matrix [6].
  • We conclude that a family of SK genes are responsible for sulfate activation in mammals, that a mutation in SK2 causes murine brachymorphism, and that members of this gene family have nonredundant, tissue-specific roles [7].

Biological context of Papss2

  • A missense mutation in the SK2 coding sequence of bm mice that alters a highly conserved amino acid residue destroys adenosine-phosphosulfate kinase activity and therefore the ability of SK2 to synthesize PAPS [7].
  • A family member, SK2, colocalizes with the locus for the autosomal recessive murine phenotype brachymorphism [7].
  • In the next step, two versions of each humanized SK2 VL and VH regions were carefully designed based on the amino acid sequences of human REI and DAW, respectively [8].
  • Previous studies have shown that the autosomal recessive gene brachymorphic (bm/bm), which is maintained on a C57BL/6J (C57) background, reduces limb growth and sulfation of cartilage proteoglycans [2].
  • In contrast, both versions of the heavy chains were comparable, in yielding good humanized SK2 antibodies, suggesting that the glycosylation of the SK2 VH region has no influence in recreating a functional antigen-binding site in this humanization [8].

Anatomical context of Papss2


Associations of Papss2 with chemical compounds

  • Proteoglycans from the brachymorphic (bm/bm) mouse have a reduced sulfate content due to the impaired activity of adenosine phosphosulfate phosphokinase in these animals [11].
  • X-ray diffraction and infrared analyses of the mineral from the calcified cartilage of the bm/bm mice demonstrate the presence of significantly larger and more perfect hydroxyapatite crystals of lower carbonate to phosphate content than crystals found in the control animals [11].
  • Morphometric analysis indicated that the time of palatal elevation was delayed in the bm/bm relative to the C57 mouse both with and without hydrocortisone treatment [2].
  • These strains differ in that elevated levels of steroid receptors are present in A/J palate, whereas lower levels are found in the C57 and bm/bm mice [2].
  • Selective suppression of SK3 expression by dietary doxycycline (DOX) decreased SK current density in isolated myocytes, increased phasic contractions of isolated urinary bladder smooth muscle strips and exposed high affinity effects of the blocker apamin of the SK isoforms (SK1-3), suggesting an additional participation from SK2 channels [12].

Other interactions of Papss2


Analytical, diagnostic and therapeutic context of Papss2

  • A biosensor study showed that the mouse SK2 Fab and its deglycosylated fragments had almost equal Kd (Kon/Koff), 26.8 nM (1.05 x 10(6)/2.81 x 10(-2)) and 24.7 nM (1.28 x 10(6)/3.15 x 10(-2)), respectively [8].
  • Rejection of H-2 class I bm 1 mutant skin allografts by B6 recipient mice is mediated by a population of CD8+ anti-bm1 cytotoxic T-lymphocytes that produces and consumes its own T helper factor in response to bm1 skin allografts (dual function CTL) [13].
  • Light microscopy, including immunohistochemical techniques, and electron microscopy were performed on epiphyseal growth cartilage from brachymorphic (bm/bm) mice and age-matched phenotypically normal siblings aged 5, 16 and 25 days [14].
  • Using immunofluorescence techniques, the presence of considerable amounts of both type II and type V collagen was demonstrated in the bm/bm mice, while the cartilage from controls contained only type II [14].


  1. Degenerative knee joint disease in mice lacking 3'-phosphoadenosine 5'-phosphosulfate synthetase 2 (Papss2) activity: a putative model of human PAPSS2 deficiency-associated arthrosis. Ford-Hutchinson, A.F., Ali, Z., Seerattan, R.A., Cooper, D.M., Hallgrímsson, B., Salo, P.T., Jirik, F.R. Osteoarthr. Cartil. (2005) [Pubmed]
  2. Cortisone-induced cleft palate in the brachymorphic mouse. Pratt, R.M., Salomon, D.S., Diewert, V.M., Erickson, R.P., Burns, R., Brown, K.S. Teratog., Carcinog. Mutagen. (1980) [Pubmed]
  3. Altered MHC class I presented peptide repertoire is not sufficient to induce NK cell mediated F1-hybrid resistance. Salcedo, M., Höglund, P., Achour, A., Thorpe, C.J., Ljunggren, H.G. Mol. Immunol. (1995) [Pubmed]
  4. Mutations in orthologous genes in human spondyloepimetaphyseal dysplasia and the brachymorphic mouse. ul Haque, M.F., King, L.M., Krakow, D., Cantor, R.M., Rusiniak, M.E., Swank, R.T., Superti-Furga, A., Haque, S., Abbas, H., Ahmad, W., Ahmad, M., Cohn, D.H. Nat. Genet. (1998) [Pubmed]
  5. Gene conversion between murine class II major histocompatibility complex loci. Functional and molecular evidence from the bm 12 mutant. Mengle-Gaw, L., Conner, S., McDevitt, H.O., Fathman, C.G. J. Exp. Med. (1984) [Pubmed]
  6. Defects in the cartilaginous growth plates of brachymorphic mice. Orkin, R.W., Williams, B.R., Cranley, R.E., Poppke, D.C., Brown, K.S. J. Cell Biol. (1977) [Pubmed]
  7. A member of a family of sulfate-activating enzymes causes murine brachymorphism. Kurima, K., Warman, M.L., Krishnan, S., Domowicz, M., Krueger, R.C., Deyrup, A., Schwartz, N.B. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  8. Humanization of an anti-human IL-6 mouse monoclonal antibody glycosylated in its heavy chain variable region. Sato, K., Ohtomo, T., Hirata, Y., Saito, H., Matsuura, T., Akimoto, T., Akamatsu, K., Koishihara, Y., Ohsugi, Y., Tsuchiya, M. Hum. Antibodies Hybridomas (1996) [Pubmed]
  9. Immunohistochemical localization of fibroblast growth factor-2 in normal and brachymorphic mouse tibial growth plate and articular cartilage. Wezeman, F.H., Bollnow, M.R. Histochem. J. (1997) [Pubmed]
  10. Sphingosine-1-phosphate signaling promotes critical migratory events in vasculogenesis. Argraves, K.M., Wilkerson, B.A., Argraves, W.S., Fleming, P.A., Obeid, L.M., Drake, C.J. J. Biol. Chem. (2004) [Pubmed]
  11. Hydroxyapatite formation in the presence of proteoglycans of reduced sulfate content: studies in the brachymorphic mouse. Boskey, A.L., Maresca, M., Wikstrom, B., Hjerpe, A. Calcif. Tissue Int. (1991) [Pubmed]
  12. Urinary bladder instability induced by selective suppression of the murine small conductance calcium-activated potassium (SK3) channel. Herrera, G.M., Pozo, M.J., Zvara, P., Petkov, G.V., Bond, C.T., Adelman, J.P., Nelson, M.T. J. Physiol. (Lond.) (2003) [Pubmed]
  13. Allograft tolerance induction in adult mice associated with functional deletion of specific CTL precursors. van Twuyver, E., Kast, W.M., Mooijaart, R.J., Wilmink, J.M., Melief, C.J., de Waal, L.P. Transplantation (1989) [Pubmed]
  14. Morphological studies of the epiphyseal growth zone in the brachymorphic (bm/bm) mouse. Wikström, B., Gay, R., Gay, S., Hjerpe, A., Mengarelli, S., Reinholt, F.P., Engfeldt, B. Virchows Arch., B, Cell Pathol. (1984) [Pubmed]
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