anti-Peripherin (PRPH) Antibodies

Human Peripherin (PRPH) interaction partners

1. Genome-wide association study of sural nerve conduction amplitude and velocity in 7045 Icelanders and find a low-frequency splice-donor variant in PRPH associated with reduced amplitude of the sural nerve action potential, but not with sural nerve conduction velocity.

2. Desmin, Glial Fibrillary Acidic Protein, Vimentin, and Peripherin are type III intermediate filaments that have roles in health and disease [review]

3. To discover of Phosphorylated autoantigens Peripherin as a Major Humoral Autoantigen in Type 1 Diabetes Mellitus.

4. In patients with HD, a panel using calretinin and peripherin with or without MAP-2 may be most helpful in identifying transition zones

5. interaction between disease-causing RAB7A mutants and peripherin could play an important role in Charcot-Marie-Tooth type 2B neuropathy

6. A protein encoded by this locus was found to be differentially expressed in postmortem brains from patients with atypical frontotemporal lobar degeneration.

7. 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.

8. 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

9. although the mechanisms underlying peripherin co-localization in Bunina bodies are unknown, peripherin could be involved in forming these inclusions

10. 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.

11. 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.

12. 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.

13. peripherin is a novel substrate for Akt in vivo and its phosphorylation may play a role in motor nerve regeneration

14. Peripherin splicing abnormalities occur in amyotrophic lateral sclerosis generating aggregation-prone splice isoforms, one of which causes peripherin aggregation when its expression is upregulated.

15. PKCepsilon through its interaction with peripherin facilitates its aggregation and subsequent cell death

16. Expression of the 2 markers, peripherin and alpha-internexin, in a small round cell tumor strongly favors the diagnosis of neuroblastoma.

17. Transgenic mice expressing the PRPH-EGFP genomic reporter display intrinsic peripheral nervous system fluorescence.

Mouse (Murine) Peripherin (PRPH) interaction partners

1. Data suggest that the underlying molecular mechanisms in amyotrophic lateral sclerosis pathogenesis involve peripherin mis-splicing and altered homeostasis of nerve fibers.

2. Contralateral and ipsilateral olivocochlear efferent-mediated suppression of the cochlear amplifier were absent in Prph null mice.

3. Data suggest anti-peripherin (PRPH) B cells represent a heterogeneous population that is generated early in life but proliferates as diabetes is established.

4. peripherin appears to be a participant in learning and memory impairment in mice

5. rather than forming an independent structure, peripherin is a subunit of neurofilaments in the adult peripheral nervous system

6. Peripherin isoform expression and inclusion formation serve a physiologically relevant role in the pathology of amyotrophic lateral sclerosis.

7. Complex post-transcriptional processes may underlie the continuum between peripherin-mediated neuronal repair and its pathogenic role in a transgenic model of amyotrophic lateral sclerosis.

8. Peripherin may interact with other cytoskeletal elements to regulate outgrowth of the peripheral neurites of type II SGN, distinguishing these neurons from the type I SGN innervating the inner hair cells.

9. peripherin gene sequences direct Cre recombinase expression to peripheral neurons in transgenic mice.

10. Overexpression of peripherin in mice deficient for neurofilament light (NF-L) subunits induced a progressive ALS-like spinal motor neuron degeneration prevented by simultaneous overexpression of NF-H

11. Of the three peripherin isoforms, Per 61 was neurotoxic, being assembly incompetent and inducing degeneration of cultured motor neurons. Per 61 was detected in motor neurons of SOD1G37R transgenic mice but not of control or peripherin transgenic mice.

12. Peripherin is not a key contributor of motor neuron degeneration associated with toxicity of mutant superoxide dismutase 1.

13. In the hippocampus of Per mice, the levels of MAP2 were decreased, though synaptophysin and PSD95 remained unchanged. These intriguing findings suggest a role of peripherin in the alteration of hippocampal synaptic plasticity.

14. mutations in peripherin/rds have roles in retinal diseases [review]

15. Mouse models on genetic macular degeneration include studies on Prph.

16. Peripherin is a major autoantigen targeted during development of type 1 diabetes mellitus; peripherin-specific B lymphocytes are mainly attracted to the islet during disease.

17. In the adult mouse hindbrain, peripherin is expressed in discrete neuronal subpopulations that have sensory, motor and autonomic functions.

18. Genetic supplementation of Prph alleviates a loss-of-function phenotype in C214S model of retinitis pigmentosa.

19. Per-45 isophorm is required for normal network formation, with the absence of Per-45 leading to irregular filamentous structures

20. Peripherin is expressed in the tuberomammillary neurons of the mouse hypothalamus.The complete overlap between histidine decarboxylase and peripherin renders peripherin a useful new marker for histaminergic neurons in the hypothalamus.