Literature DB >> 10586951

A point mutation (W70A) in the rod PDE-gamma gene desensitizing and delaying murine rod photoreceptors.

D J Salchow1, P Gouras, K Doi, S P Goff, E Schwinger, S H Tsang.   

Abstract

PURPOSE: To examine the corneal electroretinogram (ERG) of transgenic mice (W70A mice) carrying a point mutation (W70A) in the gene encoding for the gamma-subunit of rod cGMP phosphodiesterase (PDEgamma).
METHODS: The ERG of W70A mice was compared with that of normal mice. Cone responses were separated from rod responses by light adaptation, whereas rod sensitivity was assessed by threshold stimulation with dim light. Spectral sensitivity curves of the ERG were obtained using a constant response criterion.
RESULTS: The ERG of the W70A mouse has a desensitized, delayed rod b-wave at threshold, and a prolonged rod b-wave at higher flash intensities. The a-wave is absent even at maximal stimulation. The cone ERG of the W70A mouse is indistinguishable from that of normal mice. The spectral sensitivity of the W70A mouse is maximal in the UV spectrum, in contrast to the normal mouse, which is most sensitive in the green region of the spectrum. This supports the interpretation of the results as normal cone and abnormal rod function in the W70A mouse.
CONCLUSIONS: The W70A mouse represents new model of stationary nyctalopia that can be recognized by its unusual ERG features.

Entities:  

Mesh:

Substances:

Year:  1999        PMID: 10586951      PMCID: PMC2754065     

Source DB:  PubMed          Journal:  Invest Ophthalmol Vis Sci        ISSN: 0146-0404            Impact factor:   4.799


  18 in total

1.  Retinal receptors in rodents maximally sensitive to ultraviolet light.

Authors:  G H Jacobs; J Neitz; J F Deegan
Journal:  Nature       Date:  1991-10-17       Impact factor: 49.962

2.  A phosphodiesterase inhibitor specific to a subset of bovine retinal cones.

Authors:  S E Hamilton; J B Hurley
Journal:  J Biol Chem       Date:  1990-07-05       Impact factor: 5.157

3.  Mechanisms of spectral tuning in the mouse green cone pigment.

Authors:  H Sun; J P Macke; J Nathans
Journal:  Proc Natl Acad Sci U S A       Date:  1997-08-05       Impact factor: 11.205

4.  [The adaptive behavior of the retina of the mouse].

Authors:  K A Hellner
Journal:  Albrecht Von Graefes Arch Klin Exp Ophthalmol       Date:  1966-03-17

5.  Defects in the rhodopsin kinase gene in the Oguchi form of stationary night blindness.

Authors:  S Yamamoto; K C Sippel; E L Berson; T P Dryja
Journal:  Nat Genet       Date:  1997-02       Impact factor: 38.330

6.  Prolonged photoresponses in transgenic mouse rods lacking arrestin.

Authors:  J Xu; R L Dodd; C L Makino; M I Simon; D A Baylor; J Chen
Journal:  Nature       Date:  1997-10-02       Impact factor: 49.962

7.  Rod and cone function in the Nougaret form of stationary night blindness.

Authors:  M A Sandberg; B S Pawlyk; J Dan; B Arnaud; T P Dryja; E L Berson
Journal:  Arch Ophthalmol       Date:  1998-07

8.  Role for the target enzyme in deactivation of photoreceptor G protein in vivo.

Authors:  S H Tsang; M E Burns; P D Calvert; P Gouras; D A Baylor; S P Goff; V Y Arshavsky
Journal:  Science       Date:  1998-10-02       Impact factor: 47.728

9.  Loss-of-function mutations in a calcium-channel alpha1-subunit gene in Xp11.23 cause incomplete X-linked congenital stationary night blindness.

Authors:  N T Bech-Hansen; M J Naylor; T A Maybaum; W G Pearce; B Koop; G A Fishman; M Mets; M A Musarella; K M Boycott
Journal:  Nat Genet       Date:  1998-07       Impact factor: 38.330

10.  An L-type calcium-channel gene mutated in incomplete X-linked congenital stationary night blindness.

Authors:  T M Strom; G Nyakatura; E Apfelstedt-Sylla; H Hellebrand; B Lorenz; B H Weber; K Wutz; N Gutwillinger; K Rüther; B Drescher; C Sauer; E Zrenner; T Meitinger; A Rosenthal; A Meindl
Journal:  Nat Genet       Date:  1998-07       Impact factor: 38.330

View more
  9 in total

Review 1.  The retinal cGMP phosphodiesterase gamma-subunit - a chameleon.

Authors:  Lian-Wang Guo; Arnold E Ruoho
Journal:  Curr Protein Pept Sci       Date:  2008-12       Impact factor: 3.272

2.  Structural requirements of the photoreceptor phosphodiesterase gamma-subunit for inhibition of rod PDE6 holoenzyme and for its activation by transducin.

Authors:  Xiu-Jun Zhang; Nikolai P Skiba; Rick H Cote
Journal:  J Biol Chem       Date:  2009-11-30       Impact factor: 5.157

3.  Autosomal-recessive early-onset retinitis pigmentosa caused by a mutation in PDE6G, the gene encoding the gamma subunit of rod cGMP phosphodiesterase.

Authors:  Liron Dvir; Gassoub Srour; Rasmi Abu-Ras; Benjamin Miller; Stavit A Shalev; Tamar Ben-Yosef
Journal:  Am J Hum Genet       Date:  2010-07-22       Impact factor: 11.025

4.  Transplantation of reprogrammed embryonic stem cells improves visual function in a mouse model for retinitis pigmentosa.

Authors:  Nan-Kai Wang; Joaquin Tosi; Jennifer Mie Kasanuki; Chai Lin Chou; Jian Kong; Nancy Parmalee; Katherine J Wert; Rando Allikmets; Chi-Chun Lai; Chung-Liang Chien; Takayuki Nagasaki; Chyuan-Sheng Lin; Stephen H Tsang
Journal:  Transplantation       Date:  2010-04-27       Impact factor: 4.939

5.  Residual rod function in CNGB1 mutant dogs.

Authors:  Simon M Petersen-Jones; Nathaniel Pasmanter; Laurence M Occelli; Janice R Querubin; Paige A Winkler
Journal:  Doc Ophthalmol       Date:  2022-09-15       Impact factor: 1.854

6.  Functional rescue of degenerating photoreceptors in mice homozygous for a hypomorphic cGMP phosphodiesterase 6 b allele (Pde6bH620Q).

Authors:  Richard J Davis; Joaquin Tosi; Kerstin M Janisch; J Mie Kasanuki; Nan-Kai Wang; Jian Kong; Ilene Tsui; Marianne Cilluffo; Michael L Woodruff; Gordon L Fain; Chyuan-Sheng Lin; Stephen H Tsang
Journal:  Invest Ophthalmol Vis Sci       Date:  2008-07-24       Impact factor: 4.799

Review 7.  Biology and therapy of inherited retinal degenerative disease: insights from mouse models.

Authors:  Shobi Veleri; Csilla H Lazar; Bo Chang; Paul A Sieving; Eyal Banin; Anand Swaroop
Journal:  Dis Model Mech       Date:  2015-02       Impact factor: 5.758

8.  Constitutive cyclic GMP accumulation in Arabidopsis thaliana compromises systemic acquired resistance induced by an avirulent pathogen by modulating local signals.

Authors:  Jamshaid Hussain; Jian Chen; Vittoria Locato; Wilma Sabetta; Smrutisanjita Behera; Sara Cimini; Francesca Griggio; Silvia Martínez-Jaime; Alexander Graf; Mabrouk Bouneb; Raman Pachaiappan; Paola Fincato; Emanuela Blanco; Alex Costa; Laura De Gara; Diana Bellin; Maria Concetta de Pinto; Elodie Vandelle
Journal:  Sci Rep       Date:  2016-11-04       Impact factor: 4.379

Review 9.  Electrophysiological analysis of visual function in mutant mice.

Authors:  Neal S Peachey; Sherry L Ball
Journal:  Doc Ophthalmol       Date:  2003-07       Impact factor: 1.854

  9 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.