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PRPH encodes a cytoskeletal protein found in neurons of the peripheral nervous system. Additionally we are shipping Peripherin Antibodies (130) and Peripherin Kits (12) and many more products for this protein.
Showing 7 out of 8 products:
Human Peripherin Protein expressed in Wheat germ - ABIN1316403
Mizuno, Fujita, Takatama, Okamoto: Peripherin partially localizes in Bunina bodies in amyotrophic lateral sclerosis. in Journal of the neurological sciences 2011
In patients with HD, a panel using calretinin and peripherin with or without MAP-2 may be most helpful in identifying transition zones
interaction between disease-causing RAB7A (show RAB7A Proteins) mutants and peripherin could play an important role in Charcot-Marie-Tooth type 2B neuropathy
This work contributes to determine the role of PRPH gene variants in ALS. Further studies are necessary to define the mechanisms through which the mutant peripherin could cause ALS phenotype.
study analyzed expression of peripherin(PP) in the cochlea; in organ of Corti, PP seems to be specifically expressed in outer hair cell afferents; small or type II spiral ganglion cell bodies also intensely express PP
although the mechanisms underlying peripherin co-localization in Bunina bodies are unknown, peripherin could be involved in forming these inclusions
Transgenic peripherin isoform expression reveals post-transcriptional changes to the normal expression pattern associated with malformed filaments and intracellular inclusions underlying a role in the pathogenesis of amyotrophic lateral sclerosis.
The data of this experiment document the expression of peripherin in Lewy body-like inclusions , which may provide a clue to the pathogenesis of neurodegeneration in ALS.
Peripherin overexpression in transgenic mice can cause defective transport of type IV neurofilament proteins, a phenomenon that may account for the progressive formation of amyotrophic lateral sclerosis-like spheroids in axons.
peripherin is a novel substrate for Akt (show AKT1 Proteins) in vivo and its phosphorylation may play a role in motor nerve regeneration
Novel C165R mutation of retinal degeneration slow (show PRPH2 Proteins)/peripherin gene in family affected by different patterns of retinal dystrophy (show MERTK Proteins).
ablation of Rom1 (show ROM1 Proteins) results in the conversion of an MD/PD phenotype characterized by cone functional defects and the formation of abnormal Prph2 (show PRPH2 Proteins)/Rom1 (show ROM1 Proteins) complexes to an RP phenotype characterized by rod-dominant functional defects and reductions in total Prph2 (show PRPH2 Proteins) protein. Thus one method by which ROM1 (show ROM1 Proteins) may act as a disease modifier is by contributing to the large variability in PRPH2 (show PRPH2 Proteins)-associated disease phenotype
quantitative FRET analysis in acutely isolated cone OS revealed that the cone degeneration-causing V268I mutation in peripherin-2 selectively reduced binding to M-opsin without affecting the peripherin-2 interaction to S-opsin or rhodopsin
These results support the idea that mutations may differentially affect Prph2 (show PRPH2 Proteins)'s role as a structural component, and its role as a functional protein key for organizing membrane domains for cellular signalling. These roles may be different in rods and cones, thus contributing to the phenotypic heterogeneity that characterizes diseases associated with Prph2 (show PRPH2 Proteins) mutations.
Data suggest that the underlying molecular mechanisms in amyotrophic lateral sclerosis pathogenesis involve peripherin mis (show AMH Proteins)-splicing and altered homeostasis of nerve fibers.
Eliminating Cngb1 (show CNGB1 Proteins) and reducing RDS (show PRPH2 Proteins) leads to additive defects in RDS (show PRPH2 Proteins) expression levels and rod electroretinogram (ERG (show ERG Proteins)) function, (e.g., Cngb1 (show CNGB1 Proteins)-/-/rds (show PRPH2 Proteins)+/- versus rds (show PRPH2 Proteins)+/- or Cngb1 (show CNGB1 Proteins)-/-) but not to additive defects in rod ultrastructure.
In the group of mice manifesting homozygous mutation in the PRPH2 (show PRPH2 Proteins) gene.
Our data suggest that upregulation of PRPH2 (show PRPH2 Proteins) levels in combination with defects in the PRPH2 (show PRPH2 Proteins) function caused by the mutation might be an important mechanism leading to cone degeneration.
Contralateral and ipsilateral olivocochlear efferent-mediated suppression of the cochlear amplifier were absent in Prph null mice.
These data suggest that glycosylation of RDS (show PRPH2 Proteins) is required for RDS (show PRPH2 Proteins) function or stability in cones, a difference that may be due to extracellular versus intradiscal localization of the RDS (show PRPH2 Proteins) glycan in cones versus rods.
Peripherin-2 links CNGB1 to the light-detector rhodopsin in outer segments of rod photoreceptors.
This gene encodes a cytoskeletal protein found in neurons of the peripheral nervous system. The encoded protein is a type III intermediate filament protein with homology to other cytoskeletal proteins such as desmin, and is a different protein that the peripherin found in photoreceptors. Mutations in this gene have been associated with susceptibility to amyotrophic lateral sclerosis.
, peripherin 1
, neurofilament 4 (57kD)
, neuronal intermediate filament IF3
, retinal degeneration 2
, retinal degeneration slow protein
, retinal degeneration, slow (retinitis pigmentosa 7)