PMID- 31884668
OWN - NLM
STAT- MEDLINE
DCOM- 20200205
LR  - 20200205
IS  - 0065-2598 (Print)
IS  - 0065-2598 (Linking)
VI  - 1185
DP  - 2019
TI  - Studies of the Periciliary Membrane Complex in the Syrian Hamster Photoreceptor.
PG  - 543-547
LID - 10.1007/978-3-030-27378-1_89 [doi]
AB  - Mutations in USH2A, ADGRV1, and WHRN genes cause Usher syndrome type 2 (USH2) and 
      retinitis pigmentosa (RP). The proteins encoded by these genes form the 
      periciliary membrane complex (PMC) in photoreceptors. Unlike patients, who show 
      retinal degeneration in their second decade of life, mice carrying USH2 mutations 
      have very-late-onset retinal degeneration, although the PMC is disrupted. A 
      similar weak retinal degeneration phenotype was also reported in ush2a mutant 
      zebrafish. The lack of appropriate USH2 animal models hinders our understanding 
      on PMC function in photoreceptors and retinal pathogenesis caused by USH2 
      mutations. In this study, we examined the molecular composition of the PMC and 
      the morphology of the PMC and its surrounding subcellular structure in Syrian 
      hamster photoreceptors. We demonstrate that the PMC and its neighboring structure 
      in hamsters are similar to those in mice. Therefore, the Syrian hamster may not 
      offer advantages over the mouse as an animal model for USH2 pathogenic studies.
FAU - Zou, Junhuang
AU  - Zou J
AD  - Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of 
      Utah, Salt Lake City, UT, USA.
FAU - Li, Rong
AU  - Li R
AD  - Department of Animal, Dairy and Veterinary Sciences, Utah State University, 
      Logan, UT, USA.
FAU - Wang, Zhongde
AU  - Wang Z
AD  - Department of Animal, Dairy and Veterinary Sciences, Utah State University, 
      Logan, UT, USA.
FAU - Yang, Jun
AU  - Yang J
AD  - Department of Ophthalmology and Visual Sciences, Moran Eye Center, University of 
      Utah, Salt Lake City, UT, USA. jun.yang@hsc.utah.edu.
AD  - Department of Neurobiology and Anatomy, University of Utah, Salt Lake City, UT, 
      USA. jun.yang@hsc.utah.edu.
AD  - Department of Surgery, Division of Otolaryngology, University of Utah, Salt Lake 
      City, UT, USA. jun.yang@hsc.utah.edu.
LA  - eng
GR  - P30 EY014800/EY/NEI NIH HHS/United States
GR  - R01 EY020853/EY/NEI NIH HHS/United States
GR  - R01 EY026521/EY/NEI NIH HHS/United States
PT  - Journal Article
PL  - United States
TA  - Adv Exp Med Biol
JT  - Advances in experimental medicine and biology
JID - 0121103
RN  - 0 (Extracellular Matrix Proteins)
SB  - IM
MH  - Animals
MH  - Cricetinae
MH  - Disease Models, Animal
MH  - Extracellular Matrix Proteins/*genetics
MH  - Mesocricetus
MH  - Mutation
MH  - Photoreceptor Cells/*pathology
MH  - Usher Syndromes/*genetics/pathology
OTO - NOTNLM
OT  - ADGRV1
OT  - Calyceal process
OT  - Hamster
OT  - Periciliary membrane complex
OT  - Photoreceptor
OT  - Usher syndrome
OT  - Usherin
OT  - Whirlin
EDAT- 2019/12/31 06:00
MHDA- 2020/02/06 06:00
CRDT- 2019/12/30 06:00
PHST- 2019/12/30 06:00 [entrez]
PHST- 2019/12/31 06:00 [pubmed]
PHST- 2020/02/06 06:00 [medline]
AID - 10.1007/978-3-030-27378-1_89 [doi]
PST - ppublish
SO  - Adv Exp Med Biol. 2019;1185:543-547. doi: 10.1007/978-3-030-27378-1_89.