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Gene Review

Prh1  -  proline rich protein HaeIII subfamily 1

Mus musculus

Synonyms: A-type, MP2, Proline-rich protein HaeIII subfamily 1, Proline-rich protein MP-2, Prp
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Disease relevance of Prh1

  • Clones containing two nonallelic PRP genes (MP2 and M14) were isolated from cosmid and phage libraries of CD-1 mouse genomic DNA [1].
  • These results suggest that a gene or genes within the natural killer gene complex, adjacent to the Prp locus, determine strain variations in resistance to lethal ectromelia virus infection [2].
  • The association between the Prp locus and susceptibility to lethal mousepox also held for N2 male mice that were castrated as neonates, which increased the percentage that were susceptible to 40 [2].
  • Loss of intracisternal A-type retroviral particles in BL6 melanoma cells transfected with MHC class I genes [3].
  • Mutations within LMNA, encoding A-type nuclear lamins, are associated with multiple tissue-specific diseases, including Emery-Dreifuss (EDMD2/3) and Limb-Girdle muscular dystrophy (LGMD1B) [4].

High impact information on Prh1

  • By introducing mutations, we found that the three genes gag, prt and pol are all required for retrotransposition and identified the IAP gene products by electron microscopy in the form of intracellular A-type particles in the transfected cells [5].
  • These alterations are similar to those developed by mice deficient in A-type lamin, a major component of the nuclear lamina, and phenocopy most defects observed in humans with diverse congenital laminopathies [6].
  • A second A-type cyclin, cyclin A1 has been described recently which, in the mouse, is expressed in germ cells but not somatic tissues [7].
  • Here we describe the derivation of mice carrying an autosomal recessive mutation in the lamin A gene (Lmna) encoding A-type lamins, major components of the nuclear lamina [8].
  • In the brain and heart, rapidly inactivating (A-type) voltage-gated potassium (Kv) currents operate at subthreshold membrane potentials to control the excitability of neurons and cardiac myocytes [9].

Chemical compound and disease context of Prh1


Biological context of Prh1


Anatomical context of Prh1

  • Transgene expression varied among the founder lines, but SWR-like expression levels, higher than background FVB expression levels, were found in submandibular gland tissue of adult transgenic mice from two MP2 lines and one M14 line [16].
  • The discoveries of at least eight human diseases arising from mutations in LMNA, which encodes the nuclear A-type lamins, have revealed the nuclear envelope as an organelle associated with a variety of fundamental cellular processes [19].
  • Similarly, normal muscle cells that have been RNA interference (RNAi) down-regulated for either A-type lamins or emerin have impaired differentiation potentials [4].
  • In spinal cord dorsal horn neurons, A-type currents are modulated by extracellular signal-regulated kinases (ERKs), which mediate central sensitization during inflammatory pain [20].
  • CONCLUSIONS: DeltaF508 CFTR can be activated to levels 4% of wild-type when the combination of protein kinase A type II activators and phosphodiesterase class I and III inhibitors are used in murine jejunum [21].

Associations of Prh1 with chemical compounds

  • To assess the latter possibility, two type-A, proline-rich protein genes (MP2 and M14), situated approximately 30 kb apart at the Prp locus, were separately transferred from an SOA-taster inbred strain (SWR) to an SOA-nontaster inbred strain (FVB) [16].
  • Interestingly, we found that the protective effects of pituitary adenylate cyclase activating polypeptide were related to the absence of a 4-aminopyridine (IC50=144 microM) sensitive rapidly inactivating potassium current often referred to as A-type current [18].
  • Moreover, GABA, acting through A-type receptors, can both phase shift and synchronize clock cells [22].
  • Bone marrow cells were harvested after injection of 5-fluorouracil from C57BL/6-Pgk-1b/Pgk-1a female mice in which each stem cell had either A-type PGK or B-type PGK due to the random inactivation of one or two X chromosomes [23].
  • To understand how the laminopathies arise from different mutations in a single gene, we derived a mouse line by homologous recombination expressing the Lmna-N195K variant of the A-type lamins with an asparagine-to-lysine substitution at amino acid 195, which causes DCM in humans [24].

Physical interactions of Prh1

  • Calsenilin interacts with presenilins, DREAM is a calcium-regulated transcriptional repressor and KChIP3 binds and modulates A-type potassium channels [25].
  • Previous studies have indicated that A-type lamins interact with the retinoblastoma protein (pRB) [26].

Regulatory relationships of Prh1

  • Both A-type currents in cultured hippocampal neurons and A-type currents recorded from HEK 293 cells transiently expressing recombinant Kv4.2 channels were selectively inhibited by T. leblondi venom [27].
  • The AE1 gene is expressed in erythrocytes and the A-type intercalated cells of the kidney distal collecting duct [28].
  • The mechanisms of inactivation gating of the neuronal somatodendritic A-type K(+) current and the cardiac I(to) were investigated in Xenopus oocyte macropatches expressing Kv4.1 and Kv4.3 channels [29].

Other interactions of Prh1


Analytical, diagnostic and therapeutic context of Prh1

  • Restriction mapping established the physical lineage of PRP genes MP2 and M14, and they are tandemly arrayed [1].
  • We show here, by combining multiplex and quantitative real-time single-cell RT- PCR with slice patch-clamp electrophysiology, that an A-type potassium channel mediated by Kv4.3 and KChip3 subunits has a key role in pacemaker control [33].
  • Based on the 1D 1H NMR spectra, the proteins are classified into four groups, "A" to "D." A-type proteins are candidates for structure determination by NMR or crystallography; "B"-type are earmarked for crystallography; "C" indicates folded globular proteins with broadened line shapes; and "D" are nonglobular, "unfolded" polypeptides [34].
  • This is the first description of a retrovirus genome in rabbits, and sequence analysis shows that it is related to but distinct from A-type retroelements of mice and other rodents [35].
  • We used conventional voltage-clamp techniques to identify and characterize A-type currents in myocytes isolated from the murine antrum [36].


  1. Molecular evolution of the mouse proline-rich protein multigene family. Insertion of a long interspersed repeated DNA element. Ann, D.K., Smith, M.K., Carlson, D.M. J. Biol. Chem. (1988) [Pubmed]
  2. Innate resistance to lethal mousepox is genetically linked to the NK gene complex on chromosome 6 and correlates with early restriction of virus replication by cells with an NK phenotype. Delano, M.L., Brownstein, D.G. J. Virol. (1995) [Pubmed]
  3. Loss of intracisternal A-type retroviral particles in BL6 melanoma cells transfected with MHC class I genes. Li, M., Muller, J., Rao, V., Hearing, V., Lueders, K., Gorelik, E. J. Gen. Virol. (1996) [Pubmed]
  4. Lamin A/C and emerin are critical for skeletal muscle satellite cell differentiation. Frock, R.L., Kudlow, B.A., Evans, A.M., Jameson, S.A., Hauschka, S.D., Kennedy, B.K. Genes Dev. (2006) [Pubmed]
  5. Identification of autonomous IAP LTR retrotransposons mobile in mammalian cells. Dewannieux, M., Dupressoir, A., Harper, F., Pierron, G., Heidmann, T. Nat. Genet. (2004) [Pubmed]
  6. Defective prelamin A processing and muscular and adipocyte alterations in Zmpste24 metalloproteinase-deficient mice. Pendás, A.M., Zhou, Z., Cadiñanos, J., Freije, J.M., Wang, J., Hultenby, K., Astudillo, A., Wernerson, A., Rodríguez, F., Tryggvason, K., López-Otín, C. Nat. Genet. (2002) [Pubmed]
  7. Delayed early embryonic lethality following disruption of the murine cyclin A2 gene. Murphy, M., Stinnakre, M.G., Senamaud-Beaufort, C., Winston, N.J., Sweeney, C., Kubelka, M., Carrington, M., Bréchot, C., Sobczak-Thépot, J. Nat. Genet. (1997) [Pubmed]
  8. A progeroid syndrome in mice is caused by defects in A-type lamins. Mounkes, L.C., Kozlov, S., Hernandez, L., Sullivan, T., Stewart, C.L. Nature (2003) [Pubmed]
  9. Modulation of A-type potassium channels by a family of calcium sensors. An, W.F., Bowlby, M.R., Betty, M., Cao, J., Ling, H.P., Mendoza, G., Hinson, J.W., Mattsson, K.I., Strassle, B.W., Trimmer, J.S., Rhodes, K.J. Nature (2000) [Pubmed]
  10. Enhancing effect of hydrocortisone acetate administration on the content of A-type particles in Ehrlich ascites tumor. Kodama, T., Kodama, M., Nishi, Y. J. Natl. Cancer Inst. (1984) [Pubmed]
  11. PANG, a gene encoding a neuronal glycoprotein, is ectopically activated by intracisternal A-type particle long terminal repeats in murine plasmacytomas. Connelly, M.A., Grady, R.C., Mushinski, J.F., Marcu, K.B. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  12. Purification and properties of RNA-dependent DNA polymerase from cytoplasmic A-type particles of murine mammary tumor virus. Kohno, M., Ishihama, A. Eur. J. Biochem. (1979) [Pubmed]
  13. A new cell line of murine myeloid leukemia with A-type phosphoglycerate kinase as marker isoenzyme. Kawai, N., Bessho, M., Hirashima, K. Leuk. Res. (1988) [Pubmed]
  14. Diabetogenic activity of native and biosynthetic human growth hormone in obese (ob/ob) mouse. Kostyo, J.L., Gennick, S.E., Sauder, S.E. Am. J. Physiol. (1984) [Pubmed]
  15. The genetics of tasting in mice. VII. Glycine revisited, and the chromosomal location of Sac and Soa. Lush, I.E., Hornigold, N., King, P., Stoye, J.P. Genet. Res. (1995) [Pubmed]
  16. Sucrose octaacetate avoidance in nontaster mice is not enhanced by two type-A Prp transgenes from taster mice. Harder, D.B., Azen, E.A., Whitney, G. Chem. Senses (2000) [Pubmed]
  17. Localization on a physical map of the NKC-linked Cmv1 locus between Ly49b and the Prp gene cluster on mouse chromosome 6. Brown, M.G., Zhang, J., Du, Y., Stoll, J., Yokoyama, W.M., Scalzo, A.A. J. Immunol. (1999) [Pubmed]
  18. Pituitary adenylate cyclase activating polypeptide reduces A-type K+ currents and caspase activity in cultured adult mouse olfactory neurons. Han, P., Lucero, M.T. Neuroscience (2005) [Pubmed]
  19. Aging and nuclear organization: lamins and progeria. Mounkes, L.C., Stewart, C.L. Curr. Opin. Cell Biol. (2004) [Pubmed]
  20. The kv4.2 potassium channel subunit is required for pain plasticity. Hu, H.J., Carrasquillo, Y., Karim, F., Jung, W.E., Nerbonne, J.M., Schwarz, T.L., Gereau, R.W. Neuron (2006) [Pubmed]
  21. Stimulation of cystic fibrosis transmembrane conductance regulator-dependent short-circuit currents across DeltaF508 murine intestines. Steagall, W.K., Drumm, M.L. Gastroenterology (1999) [Pubmed]
  22. GABA synchronizes clock cells within the suprachiasmatic circadian clock. Liu, C., Reppert, S.M. Neuron (2000) [Pubmed]
  23. Long-term monoclonal reconstitution of erythropoiesis in genetically anemic W/Wv mice by injection of 5-fluorouracil-treated bone marrow cells of Pgk-1b/Pgk-1a mice. Nakano, T., Waki, N., Asai, H., Kitamura, Y. Blood (1987) [Pubmed]
  24. Expression of an LMNA-N195K variant of A-type lamins results in cardiac conduction defects and death in mice. Mounkes, L.C., Kozlov, S.V., Rottman, J.N., Stewart, C.L. Hum. Mol. Genet. (2005) [Pubmed]
  25. Mouse DREAM/calsenilin/KChIP3: gene structure, coding potential, and expression. Spreafico, F., Barski, J.J., Farina, C., Meyer, M. Mol. Cell. Neurosci. (2001) [Pubmed]
  26. Stabilization of the retinoblastoma protein by A-type nuclear lamins is required for INK4A-mediated cell cycle arrest. Nitta, R.T., Jameson, S.A., Kudlow, B.A., Conlan, L.A., Kennedy, B.K. Mol. Cell. Biol. (2006) [Pubmed]
  27. Modulation of Kv4.2 channels by a peptide isolated from the venom of the giant bird-eating tarantula Theraphosa leblondi. Ebbinghaus, J., Legros, C., Nolting, A., Guette, C., Celerier, M.L., Pongs, O., Bähring, R. Toxicon (2004) [Pubmed]
  28. Identification of the proximal erythroid promoter region of the mouse anion exchanger gene. Sahr, K.E., Daniels, B.P., Hanspal, M. Blood (1996) [Pubmed]
  29. Kv4 channels exhibit modulation of closed-state inactivation in inside-out patches. Beck, E.J., Covarrubias, M. Biophys. J. (2001) [Pubmed]
  30. Locus controlling Bordetella pertussis-induced histamine sensitization (Bphs), an autoimmune disease-susceptibility gene, maps distal to T-cell receptor beta-chain gene on mouse chromosome 6. Sudweeks, J.D., Todd, J.A., Blankenhorn, E.P., Wardell, B.B., Woodward, S.R., Meeker, N.D., Estes, S.S., Teuscher, C. Proc. Natl. Acad. Sci. U.S.A. (1993) [Pubmed]
  31. Prp (proline-rich protein) genes linked to markers Es-12 (esterase-12), Ea-10 (erythrocyte alloantigen), and loci on distal mouse chromosome 6. Azen, E.A., Davisson, M.T., Cherry, M., Taylor, B.A. Genomics (1989) [Pubmed]
  32. A-type lamins are essential for TGF-beta1 induced PP2A to dephosphorylate transcription factors. Van Berlo, J.H., Voncken, J.W., Kubben, N., Broers, J.L., Duisters, R., van Leeuwen, R.E., Crijns, H.J., Ramaekers, F.C., Hutchison, C.J., Pinto, Y.M. Hum. Mol. Genet. (2005) [Pubmed]
  33. Tuning pacemaker frequency of individual dopaminergic neurons by Kv4.3L and KChip3.1 transcription. Liss, B., Franz, O., Sewing, S., Bruns, R., Neuhoff, H., Roeper, J. EMBO J. (2001) [Pubmed]
  34. NMR screening and crystal quality of bacterially expressed prokaryotic and eukaryotic proteins in a structural genomics pipeline. Page, R., Peti, W., Wilson, I.A., Stevens, R.C., Wüthrich, K. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  35. Novel endogenous retrovirus in rabbits previously reported as human retrovirus 5. Griffiths, D.J., Voisset, C., Venables, P.J., Weiss, R.A. J. Virol. (2002) [Pubmed]
  36. Characterization of the A-type potassium current in murine gastric antrum. Amberg, G.C., Baker, S.A., Koh, S.D., Hatton, W.J., Murray, K.J., Horowitz, B., Sanders, K.M. J. Physiol. (Lond.) (2002) [Pubmed]
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