AIPL1, a protein implicated in Leber's congenital amaurosis, interacts with and aids in processing of farnesylated proteins
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AIPL1, the protein that is defective in Leber congenital amaurosis, is essential for the biosynthesis of retinal rod cGMP phosphodiesteraseLeber congenital amaurosis linked to AIPL1: a mouse model reveals destabilization of cGMP phosphodiesteraseExtended conformation of the proline-rich domain of human aryl hydrocarbon receptor-interacting protein-like 1: implications for retina disease.Intensification: A Resource for Amplifying Population-Genetic Signals with Protein Repeats.Enlarged meristems and delayed growth in plp mutants result from lack of CaaX prenyltransferases.Gene therapy with a promoter targeting both rods and cones rescues retinal degeneration caused by AIPL1 mutationsThe Leber congenital amaurosis protein, AIPL1, is needed for the viability and functioning of cone photoreceptor cellsLeber congenital amaurosis associated with AIPL1: challenges in ascribing disease causation, clinical findings, and implications for gene therapy.The Leber congenital amaurosis protein AIPL1 modulates the nuclear translocation of NUB1 and suppresses inclusion formation by NUB1 fragments.RAS-converting enzyme 1-mediated endoproteolysis is required for trafficking of rod phosphodiesterase 6 to photoreceptor outer segmentsRoles of glucose in photoreceptor survival.Residual electroretinograms in young Leber congenital amaurosis patients with mutations of AIPL1Deletion of PrBP/delta impedes transport of GRK1 and PDE6 catalytic subunits to photoreceptor outer segments.Greasing the protein biosynthesis machinery of photoreceptor neurons: Role for postprenylation processing of proteins.Genetic disorders involving molecular-chaperone genes: a perspective.Phosphodiesterase 6β Expression In Developing Mouse Retina.Characterization of a canine model of autosomal recessive retinitis pigmentosa due to a PDE6A mutation.New mouse models for recessive retinitis pigmentosa caused by mutations in the Pde6a geneChemical approaches to mapping the function of post-translational modifications.Molecular chaperones and photoreceptor function.Interaction of aryl hydrocarbon receptor-interacting protein-like 1 with the farnesyl moietyGene therapy for retinitis pigmentosa and Leber congenital amaurosis caused by defects in AIPL1: effective rescue of mouse models of partial and complete Aipl1 deficiency using AAV2/2 and AAV2/8 vectorsAIPL1, a protein associated with childhood blindness, interacts with alpha-subunit of rod phosphodiesterase (PDE6) and is essential for its proper assembly.AIPL1, A protein linked to blindness, is essential for the stability of enzymes mediating cGMP metabolism in cone photoreceptor cells.Prenylation defects in inherited retinal diseases.Leber congenital amaurosis, from darkness to light: An ode to Irene Maumenee.The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex.Imbalances in the Hsp90 Chaperone Machinery: Implications for Tauopathies.The integrity and organization of the human AIPL1 functional domains is critical for its role as a HSP90-dependent co-chaperone for rod PDE6.AIPL1: A specialized chaperone for the phototransduction effector.Mechanisms of mutant PDE6 proteins underlying retinal diseases.NMR resonance assignments of the FKBP domain of human aryl hydrocarbon receptor-interacting protein-like 1 (AIPL1) in complex with a farnesyl ligand.
P2860
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P2860
AIPL1, a protein implicated in Leber's congenital amaurosis, interacts with and aids in processing of farnesylated proteins
description
2003 nî lūn-bûn
@nan
2003 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2003年の論文
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2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@ast
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@en
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@nl
type
label
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@ast
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@en
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@nl
prefLabel
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@ast
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@en
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@nl
P2093
P2860
P921
P356
P1476
AIPL1, a protein implicated in ...... ssing of farnesylated proteins
@en
P2093
Gregory Niemi
James B Hurley
Melanie Roberts
Visvanathan Ramamurthy
P2860
P304
P356
10.1073/PNAS.2134194100
P407
P577
2003-10-28T00:00:00Z