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@ARTICLE{Iannaccone:54920,
author = {Iannaccone, A. and Man, D. and Waseem, N. and Jennings, B.
J. and Ganapathiraju, M. and Gallaher, K. and Reese, E. and
Bhattacharya, S. S. and Klein-Seetharaman, J.},
title = {{R}etinitis {P}igmentosa {A}sssociated with {R}hodopsin
{M}utations: {C}orrelation between {P}henotypic
{V}ariability and {M}olecular {E}ffects.},
journal = {Vision research},
volume = {46},
issn = {0042-6989},
address = {Amsterdam [u.a.]},
publisher = {Elsevier Science},
reportid = {PreJuSER-54920},
pages = {4556 - 4567},
year = {2006},
note = {Record converted from VDB: 12.11.2012},
abstract = {Similar retinitis pigmentosa (RP) phenotypes can result
from mutations affecting different rhodopsin regions, and
distinct amino acid substitutions can cause different RP
severity and progression rates. Specifically, both the R135L
and R135W mutations (cytoplasmic end of H3) result in
diffuse, severe disease (class A), but R135W causes more
severe and more rapidly progressive RP than R135L. The P180A
and G188R mutations (second intradiscal loop) exhibit a mild
phenotype with regional variability (class B1) and diffuse
disease of moderate severity (class B2), respectively.
Computational and in vitro studies of these mutants provide
molecular insights into this phenotypic variability.},
keywords = {Adolescent / Adult / Age Factors / Amino Acid Substitution
/ Child / Child, Preschool / Computational Biology / DNA
Mutational Analysis / Disease Progression /
Electroretinography / Female / Humans / Male / Mutation /
Pedigree / Peptide Fragments: genetics / Phenotype /
Retinitis Pigmentosa: genetics / Retinitis Pigmentosa:
metabolism / Rhodopsin: genetics / Rhodopsin: metabolism /
Rod Cell Outer Segment: metabolism / Vision, Ocular /
Peptide Fragments (NLM Chemicals) / Rhodopsin (NLM
Chemicals) / J (WoSType)},
cin = {IBI-2},
ddc = {610},
cid = {I:(DE-Juel1)VDB58},
pnm = {Funktion und Dysfunktion des Nervensystems},
pid = {G:(DE-Juel1)FUEK409},
shelfmark = {Neurosciences / Ophthalmology},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:17014888},
UT = {WOS:000242674400015},
doi = {10.1016/j.visres.2006.08.018},
url = {https://juser.fz-juelich.de/record/54920},
}