Accumulation of non-outer segment proteins in the outer segment underlies photoreceptor degeneration in Bardet-Biedl syndrome.
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Whole organism transcriptome analysis of zebrafish models of Bardet-Biedl Syndrome and Alström Syndrome provides mechanistic insight into shared and divergent phenotypes.ARL3 regulates trafficking of prenylated phototransduction proteins to the rod outer segment.An Actin Network Dispatches Ciliary GPCRs into Extracellular Vesicles to Modulate Signaling.Molecular basis for photoreceptor outer segment architecture.Protein transport in growing and steady-state cilia.Gene Therapeutic Reversal of Peripheral Olfactory Impairment in Bardet-Biedl Syndrome.Primary Cilia and Coordination of Receptor Tyrosine Kinase (RTK) and Transforming Growth Factor β (TGF-β) Signaling.Photoreceptor outer segment as a sink for membrane proteins: hypothesis and implications in retinal ciliopathies.From compliment to insult: genetics of the complement system in physiology and disease in the human retina.Bardet-Biedl Syndrome -8 (BBS8) protein is crucial for the development of outer segments in photoreceptor neurons.BBSome function is required for both the morphogenesis and maintenance of the photoreceptor outer segment.Loss-of-function of the ciliopathy protein Cc2d2a disorganizes the vesicle fusion machinery at the periciliary membrane and indirectly affects Rab8-trafficking in zebrafish photoreceptors.The Arf GEF GBF1 and Arf4 synergize with the sensory receptor cargo, rhodopsin, to regulate ciliary membrane trafficking.The Bardet-Biedl syndrome protein complex is an adapter expanding the cargo range of intraflagellar transport trains for ciliary export.Farnesylation of the Transducin G Protein Gamma Subunit Is a Prerequisite for Its Ciliary Targeting in Rod Photoreceptors.Keeping an Eye on Bardet-Biedl Syndrome: A Comprehensive Review of the Role of Bardet-Biedl Syndrome Genes in the Eye.The molecular machines that traffic signaling receptors into and out of cilia.BBSome trains remove activated GPCRs from cilia by enabling passage through the transition zone.ARL2BP, a protein linked to Retinitis Pigmentosa, is needed for normal photoreceptor cilia doublets and outer segment structure.
P2860
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P2860
Accumulation of non-outer segment proteins in the outer segment underlies photoreceptor degeneration in Bardet-Biedl syndrome.
description
2015 nî lūn-bûn
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2015年の論文
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2015年論文
@yue
2015年論文
@zh-hant
2015年論文
@zh-hk
2015年論文
@zh-mo
2015年論文
@zh-tw
2015年论文
@wuu
2015年论文
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2015年论文
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name
Accumulation of non-outer segm ...... tion in Bardet-Biedl syndrome.
@ast
Accumulation of non-outer segm ...... tion in Bardet-Biedl syndrome.
@en
type
label
Accumulation of non-outer segm ...... tion in Bardet-Biedl syndrome.
@ast
Accumulation of non-outer segm ...... tion in Bardet-Biedl syndrome.
@en
prefLabel
Accumulation of non-outer segm ...... tion in Bardet-Biedl syndrome.
@ast
Accumulation of non-outer segm ...... tion in Bardet-Biedl syndrome.
@en
P2093
P2860
P50
P356
P1476
Accumulation of non-outer segm ...... ation in Bardet-Biedl syndrome
@en
P2093
Chantal Allamargot
Emily K Andersen
Joseph S Hudson
Sajag Bhattarai
P2860
P304
P356
10.1073/PNAS.1510111112
P407
P577
2015-07-27T00:00:00Z