The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex. - Test2 - elhamkhani - TriplyDB

The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex.

The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex.

http://www.wikidata.org/entity/Q39992486

about

Hsp90 as a Potential Therapeutic Target in Retinal Disease Comprehensive genotyping reveals RPE65 as the most frequently mutated gene in Leber congenital amaurosis in Denmark Extended conformation of the proline-rich domain of human aryl hydrocarbon receptor-interacting protein-like 1: implications for retina disease.Hsp90 and co-chaperones twist the functions of diverse client proteins.The inherited blindness protein AIPL1 regulates the ubiquitin-like FAT10 pathway Leber congenital amaurosis associated with AIPL1: challenges in ascribing disease causation, clinical findings, and implications for gene therapy.Differential proteomics and functional research following gene therapy in a mouse model of Leber congenital amaurosis The Leber congenital amaurosis protein AIPL1 and EB proteins co-localize at the photoreceptor cilium Characterization of aryl hydrocarbon receptor interacting protein (AIP) mutations in familial isolated pituitary adenoma families.Synphilin-1-binding protein NUB1 is colocalized with nonfibrillar, proteinase K-resistant α-synuclein in presynapses in Lewy body disease Residual electroretinograms in young Leber congenital amaurosis patients with mutations of AIPL1 Characterization of a canine model of autosomal recessive retinitis pigmentosa due to a PDE6A mutation.Molecular chaperones and photoreceptor function.Interaction of aryl hydrocarbon receptor-interacting protein-like 1 with the farnesyl moiety NEDD8 ultimate buster-1 long (NUB1L) protein promotes transfer of NEDD8 to proteasome for degradation through the P97UFD1/NPL4 complex.AIPL1, a protein associated with childhood blindness, interacts with alpha-subunit of rod phosphodiesterase (PDE6) and is essential for its proper assembly.Hsp90 inhibition protects against inherited retinal degeneration.Leber congenital amaurosis/early-onset severe retinal dystrophy: clinical features, molecular genetics and therapeutic interventions.Mouse models of human ocular disease for translational research.Aryl Hydrocarbon Receptor-interacting Protein-like 1 Is an Obligate Chaperone of Phosphodiesterase 6 and Is Assisted by the γ-Subunit of Its Client.Prevention of autosomal dominant retinitis pigmentosa by systemic drug therapy targeting heat shock protein 90 (Hsp90).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.Unique structural features of the AIPL1-FKBP domain that support prenyl lipid binding and underlie protein malfunction in blindness.

P2860

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P2860

The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex.

http://www.wikidata.org/entity/Q39992486

description

2008 nî lūn-bûn

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2008年の論文

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2008年学术文章

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2008年学术文章

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2008年学术文章

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2008年学术文章

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2008年学术文章

@zh-sg

2008年學術文章

@yue

2008年學術文章

@zh

2008年學術文章

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name

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@en

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@nl

type

label

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@en

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@nl

prefLabel

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@en

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@nl

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Investigative Ophthalmology & Visual Science

P1476

The Leber congenital amaurosis ...... of a chaperone heterocomplex.

@en

P2093

Jacqueline van der Spuy

http://www.w3.org/2001/XMLSchema#string

Juan Hidalgo-de-Quintana

http://www.w3.org/2001/XMLSchema#string

Michael E Cheetham

http://www.w3.org/2001/XMLSchema#string

R Jane Evans

http://www.w3.org/2001/XMLSchema#string

P2860

Mutations in a new photoreceptor-pineal gene on 17p cause Leber congenital amaurosis

Binding of aryl hydrocarbon receptor (AhR) to AhR-interacting protein - The role of hsp90

Functional analysis of the Hsp90-associated human peptidyl prolyl cis/trans isomerases FKBP51, FKBP52 and Cyp40

AIPL1, a protein implicated in Leber's congenital amaurosis, interacts with and aids in processing of farnesylated proteins

AIP is a mitochondrial import mediator that binds to both import receptor Tom20 and preproteins

A structure-based mutational analysis of cyclophilin 40 identifies key residues in the core tetratricopeptide repeat domain that mediate binding to Hsp90

NEDD8 ultimate buster-1L interacts with the ubiquitin-like protein FAT10 and accelerates its degradation

The inherited blindness associated protein AIPL1 interacts with the cell cycle regulator protein NUB1

Specific binding of tetratricopeptide repeat proteins to the C-terminal 12-kDa domain of hsp90

Ligand-dependent interaction of the aryl hydrocarbon receptor with a novel immunophilin homolog in vivo

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2878-2887

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scholarly article

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10.1167/IOVS.07-1576

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7

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49

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2008-04-11T00:00:00Z

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5444863

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18408180

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molecular chaperones

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2488389

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