Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations.

Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Research Abstract Details 

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Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Abstract Text:

Andrew F X Goldberg,

The vertebrate photoreceptor outer segment (OS) is a highly structured and dynamic organelle specialized to transduce light signals. The elaborate membranous architecture of the OS requires peripherin/rds (P/rds), an integral membrane protein and tetraspanin protein family member. Gene-level defects in P/rds cause a broad variety of late-onset progressive retinal degenerations in humans and dysmorphic photoreceptors in murine and Xenopus models. Although proposed to fulfill numerous roles related to OS structural stability and renewal, P/rds molecular function remains uncertain. An increasingly resolved model of this protein's oligomeric structure can account for disease inheritance patterns and severity in some instances. Nonetheless, the pathogenic mechanisms underlying the uniquely broad spectrum of retinal diseases associated with P/rds defects are not currently well understood. Recent findings point to the possibility that P/rds acts as a multifunctional scaffolding protein for OS architecture and that partial-loss-of-function mutations contribute to the hallmark phenotypic heterogeneity associated with inherited defects in RDS.

Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Publishing Authors By Initials

AF Goldberg,

For similar biochemical phenomena, metabolism, and nutrition: biochemical phenomena: sequence homology: sequence homology, amino acid research abstracts see: biochemical phenomena, metabolism, and nutrition: biochemical phenomena: sequence homology: sequence homology, amino acid research

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Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Journal Published:

PUBLICATION TYPE: Review

Journal: International review of cytology

VOLUME: 253

Page Numbers: 131-75

Journal Abbreviation: Int. Rev. Cytol.

ISSN: 0074-7696

DAY: 3

MONTH: 12

YEAR: 2006

Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 2985180

Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Keywords Mesh Terms:

KEYWORDS: Sequence Homology, Amino Acid

MESH TERMS: genetics

Chemical & Substance for Abstract: Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations. Information

Substance Name: peripherin

Registry Number: 0

Grant and Affiliation Information for Role of peripherin/rds in vertebrate photoreceptor architecture and inherited retinal degenerations.

AFFILIATION: Eye Research Institute, Oakland University, Rochester, Michigan 48309, USA.

Country: United States

AGENCY: United States NCRR

GRANT: RR17890

ACRONYM: RR

MEDLINETA: Int Rev Cytol

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