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Gene Review

Pde6h  -  phosphodiesterase 6H, cGMP-specific, cone,...

Mus musculus

Synonyms: A930033D18Rik, GMP-PDE gamma, PDEgamma, Retinal cone rhodopsin-sensitive cGMP 3',5'-cyclic phosphodiesterase subunit gamma
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Disease relevance of Pde6h


High impact information on Pde6h


Biological context of Pde6h

  • However, the Y84G mutation did not fully eliminate the control of PDEgamma on the PDE catalytic core in vivo; the expression of the mutant subunit was associated with only a 10-fold reduction in the amplitude of the a-wave and a 1.5-fold decrease in the b-wave of the corneal electroretinogram [5].
  • Here we describe the morphology and physiology of the retina in mice carrying four different Pdeg alleles: Pdeg(tm), Del 7C, Y84G, and W70A and the effect that these mutations of PDE gamma have on components of the activation and deactivation phases of phototransduction [1].
  • A point mutation (W70A) in the rod PDE-gamma gene desensitizing and delaying murine rod photoreceptors [6].
  • Comparison of the deduced amino acid sequences of the proteins from the three species indicates that PDE gamma has been very well conserved through evolution [7].

Anatomical context of Pde6h

  • Here, we report that mouse lung expresses gamma-subunit (PDEgamma) transcripts of the rod and cone photoreceptor cGMP phosphodiesterase genes (Pde6g and Pde6h, respectively) [8].
  • Second, the expression of p14 immunostained with antibodies recognizing both rod and cone PDEgamma was substantially reduced in lung membranes from Pde6g-/- mice [8].
  • 2. We show for the first time that PDEgamma is expressed in rat main, first, intrapulmonary and resistance pulmonary arteries [3].

Physical interactions of Pde6h

  • Immobilized PDE gamma binds transducin alpha subunit charged with GTP, PDE alpha and beta subunits, and, unexpectedly, arrestin (S-antigen) [9].

Regulatory relationships of Pde6h

  • The addition of rod PDEgamma to the incubation completely blocked the cleavage of PDE6 by caspase-3 [10].

Other interactions of Pde6h


Analytical, diagnostic and therapeutic context of Pde6h


  1. Stationary night blindness or progressive retinal degeneration in mice carrying different alleles of PDE gamma. Farber, D.B., Tsang, S.H. Front. Biosci. (2003) [Pubmed]
  2. Ribozyme knockdown of the gamma-subunit of rod cGMP phosphodiesterase alters the ERG and retinal morphology in wild-type mice. Liu, J., Timmers, A.M., Lewin, A.S., Hauswirth, W.W. Invest. Ophthalmol. Vis. Sci. (2005) [Pubmed]
  3. An assessment of the role of the inhibitory gamma subunit of the retinal cyclic GMP phosphodiesterase and its effect on the p42/p44 mitogen-activated protein kinase pathway in animal and cellular models of pulmonary hypertension. Murray, F., MacLean, M.R., Pyne, N.J. Br. J. Pharmacol. (2003) [Pubmed]
  4. The inhibitory gamma subunit of the type 6 retinal cyclic guanosine monophosphate phosphodiesterase is a novel intermediate regulating p42/p44 mitogen-activated protein kinase signaling in human embryonic kidney 293 cells. Wan, K.F., Sambi, B.S., Frame, M., Tate, R., Pyne, N.J. J. Biol. Chem. (2001) [Pubmed]
  5. In vivo studies of the gamma subunit of retinal cGMP-phophodiesterase with a substitution of tyrosine-84. Tsang, S.H., Yamashita, C.K., Doi, K., Salchow, D.J., Bouvier, N., Mendelsohn, M., Gouras, P., Farber, D.B., Goff, S.P. Biochem. J. (2001) [Pubmed]
  6. A point mutation (W70A) in the rod PDE-gamma gene desensitizing and delaying murine rod photoreceptors. Salchow, D.J., Gouras, P., Doi, K., Goff, S.P., Schwinger, E., Tsang, S.H. Invest. Ophthalmol. Vis. Sci. (1999) [Pubmed]
  7. Isolation and characterization of cDNA encoding the gamma-subunit of cGMP phosphodiesterase in human retina. Tuteja, N., Danciger, M., Klisak, I., Tuteja, R., Inana, G., Mohandas, T., Sparkes, R.S., Farber, D.B. Gene (1990) [Pubmed]
  8. The identification of the inhibitory gamma-subunits of the type 6 retinal cyclic guanosine monophosphate phosphodiesterase in non-retinal tissues: differential processing of mRNA transcripts. Tate, R.J., Arshavsky, V.Y., Pyne, N.J. Genomics (2002) [Pubmed]
  9. Expression of mouse rod photoreceptor cGMP phosphodiesterase gamma subunit in bacteria. Qin, N., Baehr, W. FEBS Lett. (1993) [Pubmed]
  10. The gamma subunit of the rod photoreceptor cGMP phosphodiesterase can modulate the proteolysis of two cGMP binding cGMP-specific phosphodiesterases (PDE6 and PDE5) by caspase-3. Frame, M., Wan, K.F., Tate, R., Vandenabeele, P., Pyne, N.J. Cell. Signal. (2001) [Pubmed]
  11. Cloning and characterization of the cDNA and gene encoding the gamma-subunit of cGMP-phosphodiesterase in canine retinal rod photoreceptor cells. Wang, W., Acland, G.M., Aguirre, G.D., Ray, K. Gene (1996) [Pubmed]
  12. Retinal degeneration in mice lacking the gamma subunit of the rod cGMP phosphodiesterase. Tsang, S.H., Gouras, P., Yamashita, C.K., Kjeldbye, H., Fisher, J., Farber, D.B., Goff, S.P. Science (1996) [Pubmed]
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