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Prph  -  peripherin

Mus musculus

Synonyms: Peripherin, Prph1
 
 
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Disease relevance of Prph1

 

High impact information on Prph1

 

Biological context of Prph1

  • Defective NF transport by peripherin up-regulation was further confirmed with dorsal root ganglia (DRG) neurons cultured from peripherin transgenic embryos [1].
  • Here, we show that peripherin overexpression slows down axonal transport of neurofilament (NF) proteins, and that the transport defect precedes by several months the appearance of axonal spheroids in adult mice [1].
  • These results indicate that AP-2alpha is not essential for the separation of the olfactory and respiratory epithelium from the nasal placode and is consistent with AP-2alpha preventing recapitulation of developmental programs within the respiratory epithelium that lead to expression of LHRH and peripherin phenotypes [9].
  • This was done by the generation and analysis of SOD1(G37R) mice that either overexpress a peripherin transgene (G37R;TgPer mice) or lack the endogenous peripherin gene (G37R;Per-/- mice) [3].
  • In the present work, we performed an in vitro functional analysis of the 5' flanking region of the mouse peripherin gene and observed that this region up to 9 kb contained both enhancer and inhibiting activities; however, it was insufficient to achieve a complete extinction of reporter gene expression in peripherin-negative cells [10].
 

Anatomical context of Prph1

 

Associations of Prph1 with chemical compounds

  • Acetylcholine esterase and peripherin mRNA level decrease in wobbler mouse [11].
  • Here, we report a similar pattern of peripherin expression in the brains of both mice treated with systemic injections of kainic acid (KA) and in peripherin transgenic mice (Per mice) over-expressing the normal peripherin gene under its own promoter [12].
  • Serotonin was present in trigeminal neurons containing CGRP, a potent vasoactive neuropeptide, peripherin, an intermediate filament present in neurons with unmyelinated axons, neurofilament H, which is present in neurons with myelinated axons, and in neurons binding IB4, a marker of nonpeptidergic nociceptors [13].
 

Co-localisations of Prph1

 

Regulatory relationships of Prph1

 

Other interactions of Prph1

 

Analytical, diagnostic and therapeutic context of Prph1

References

  1. Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin. Millecamps, S., Robertson, J., Lariviere, R., Mallet, J., Julien, J.P. J. Neurochem. (2006) [Pubmed]
  2. Multiple mRNAs encode peripherin, a neuronal intermediate filament protein. Landon, F., Lemonnier, M., Benarous, R., Huc, C., Fiszman, M., Gros, F., Portier, M.M. EMBO J. (1989) [Pubmed]
  3. Peripherin is not a contributing factor to motor neuron disease in a mouse model of amyotrophic lateral sclerosis caused by mutant superoxide dismutase. Larivière, R.C., Beaulieu, J.M., Nguyen, M.D., Julien, J.P. Neurobiol. Dis. (2003) [Pubmed]
  4. Cell type-specific expression of the mouse peripherin gene requires both upstream and intragenic sequences in transgenic mouse embryos. Leconte, L., Santha, M., Fort, C., Poujeol, C., Portier, M.M., Simonneau, M. Brain Res. Dev. Brain Res. (1996) [Pubmed]
  5. Origin of the beta cells of the islets of Langerhans is further questioned by the expression of neuronal intermediate filament proteins, peripherin and NF-L, in the rat insulinoma RIN5F cell line. Escurat, M., Djabali, K., Huc, C., Landon, F., Bécourt, C., Boitard, C., Gros, F., Portier, M.M. Dev. Neurosci. (1991) [Pubmed]
  6. A neurotoxic peripherin splice variant in a mouse model of ALS. Robertson, J., Doroudchi, M.M., Nguyen, M.D., Durham, H.D., Strong, M.J., Shaw, G., Julien, J.P., Mushynski, W.E. J. Cell Biol. (2003) [Pubmed]
  7. The intermediate filament protein peripherin is the specific interaction partner of mouse BPAG1-n (dystonin) in neurons. Leung, C.L., Sun, D., Liem, R.K. J. Cell Biol. (1999) [Pubmed]
  8. Late onset of motor neurons in mice overexpressing wild-type peripherin. Beaulieu, J.M., Nguyen, M.D., Julien, J.P. J. Cell Biol. (1999) [Pubmed]
  9. Ectopic expression of luteinizing hormone-releasing hormone and peripherin in the respiratory epithelium of mice lacking transcription factor AP-2alpha. Kramer, P.R., Guerrero, G., Krishnamurthy, R., Mitchell, P.J., Wray, S. Mech. Dev. (2000) [Pubmed]
  10. Involvement of intronic sequences in cell-specific expression of the peripherin gene. Lecomte, M.J., Basseville, M., Fauquet, M. J. Neurochem. (1999) [Pubmed]
  11. Acetylcholine esterase and peripherin mRNA level decrease in wobbler mouse. Pernas-Alonso, R., Schaffner, A.E., Hansen, C.T., Barker, J.L., di Porzio, U. Neuroreport (1995) [Pubmed]
  12. Up-regulation of peripherin is associated with alterations in synaptic plasticity in CA1 and CA3 regions of hippocampus. Kriz, J., Beaulieu, J.M., Julien, J.P., Krnjević, K. Neurobiol. Dis. (2005) [Pubmed]
  13. Serotonin in trigeminal Ganglia of female rodents: relevance to menstrual migraine. Berman, N.E., Puri, V., Chandrala, S., Puri, S., Macgregor, R., Liverman, C.S., Klein, R.M. Headache. (2006) [Pubmed]
  14. Distribution of neuronal intermediate filament proteins in the developing mouse olfactory system. Chien, C.L., Lee, T.H., Lu, K.S. J. Neurosci. Res. (1998) [Pubmed]
  15. A subset of peripherin positive olfactory axons delineates the luteinizing hormone releasing hormone neuronal migratory pathway in developing mouse. Wray, S., Key, S., Qualls, R., Fueshko, S.M. Dev. Biol. (1994) [Pubmed]
  16. Analysis of mice carrying targeted mutations of the glucocorticoid receptor gene argues against an essential role of glucocorticoid signalling for generating adrenal chromaffin cells. Finotto, S., Krieglstein, K., Schober, A., Deimling, F., Lindner, K., Brühl, B., Beier, K., Metz, J., Garcia-Arraras, J.E., Roig-Lopez, J.L., Monaghan, P., Schmid, W., Cole, T.J., Kellendonk, C., Tronche, F., Schütz, G., Unsicker, K. Development (1999) [Pubmed]
  17. LHRH cells migrate on peripherin fibers in embryonic olfactory explant cultures: an in vitro model for neurophilic neuronal migration. Fueshko, S., Wray, S. Dev. Biol. (1994) [Pubmed]
  18. Murine peripherin gene sequences direct Cre recombinase expression to peripheral neurons in transgenic mice. Zhou, L., Népote, V., Rowley, D.L., Levacher, B., Zvara, A., Santha, M., Mi, Q.S., Simonneau, M., Donovan, D.M. FEBS Lett. (2002) [Pubmed]
  19. Chromosomal localisation of the mouse and human peripherin genes. Moncla, A., Landon, F., Mattei, M.G., Portier, M.M. Genet. Res. (1992) [Pubmed]
 
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