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Gene Review

RERE  -  arginine-glutamic acid dipeptide (RE) repeats

Homo sapiens

Synonyms: ARG, ARP, ATN1L, Arginine-glutamic acid dipeptide repeats protein, Atrophin-1-like protein, ...
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Disease relevance of RERE

  • In particular, further studies of the RERE gene and its products in HCV associated liver disease are warranted [1].
  • Here we show that neuroblastoma tumor cell lines display reduced abundance of RERE transcripts [2].
  • Our initial studies using a microtiter assay show that CAV exhibits a 50% inhibitory concentration of approximately 2 mM against the human pancreatic adenocarcinoma cell line, MIA PaCa-2, when these cells are grown in Dulbecco's modified Eagle's medium containing 0.4 mM ARG [3].
  • The median peak GH response to ARG was significantly lower in the patients with idiopathic inflammatory pituitary stalk thickness (1.3 microg/liter; range, 0.8-1.8) than in those with craniopharyngioma (1.5 microg/liter; range, 1.1-1.6) or with LCH (2.8 microg/liter; range, 1.9-3.2, P = 0.00007) [4].
  • This study aimed at evaluating the effects of ARG on the NO system and renal function in proteinuric patients with moderate chronic renal failure (CRF) [5].

Psychiatry related information on RERE

  • In contrast to the PD + GHRH test, the ARG + GHRH test is reliable throughout the adult lifespan and appears to be the most appropriate for patient compliance and safety [6].

High impact information on RERE

  • More RERE protein is recruited into nuclear aggregates of the DRPLA protein with extended polyglutamine than into those of pure polyglutamine [7].
  • The gene isolated, designated RERE, has an open reading frame of 1566 amino acids, of which the C-terminal portion has 67% homology to DRPLA, whereas the N-terminal portion is distinctive [7].
  • The ETV6/ARG protein may be involved in the unique differentiation capacity of this cell line [8].
  • L-Canavanine (CAV), the L-2-amino-4-guanidinooxy structural analogue of L-arginine (ARG), is a potent ARG antagonist which occurs in the jack bean, Canavalia ensiformis [3].
  • The pronounced increase in the ability of CAV to inhibit MIA PaCa-2 cell growth at the lower ARG concentration may result from enhanced CAV competition with ARG for incorporation into newly synthesized cellular proteins [3].

Chemical compound and disease context of RERE


Biological context of RERE


Anatomical context of RERE

  • FcR number and avidity were determined from Scatchard analysis of binding of 125I-labeled aggregated rat IgG (ARG) to macrophages [14].
  • Physiologically relevant ARP26)levels promoted AChE gene expression and induced the expansion of cultured CD34+ progenitors and granulocyte maturation more effectively than cortisol, suggesting autoregulatory prolongation of ARP effects [15].
  • MATERIALS AND METHODS: We studied the effects of stress, cortisol, antisense oligonucleotides to AChE, and synthetic ARP on peripheral blood cell composition and clonogenic progenitor status in mice under normal and stress conditions, and on purified CD34 cells of human origin [16].
  • Ex vivo, ARP was more effective than cortisol and equally as effective as stem cell factor in promoting expansion and differentiation of early hematopoietic progenitor cells into myeloid and megakaryocyte lineages [16].
  • In conclusion, plasma ARG concentrations and systemic NO synthesis were not deficient in preterm infants with BPD and signs of elevated pulmonary artery pressure [17].

Associations of RERE with chemical compounds

  • RERE also contains arginine-aspartic acid (RD) dipeptide repeats and putative nuclear localization signal sequences, but no polyglutamine tracts [7].
  • A major product of this process is a large nonaggregating species that consists of an NH2-terminal sequence beginning with ARG (and composed of about 100 residues of the interglobular domain) that is attached to an intact G2 domain followed by an extended section of the chondroitin sulfate-bearing domain toward the COOH terminus [18].
  • Therefore, only GHRH combined with PD or ARG may be able to clearly differentiate normal children from patients with GH deficiency, though a normal GH response to these tests cannot rule out the existence of GH hyposecretory state because of hypothalamic dysfunction [19].
  • During the six months of treatment, although a remarkable steadiness of ARG and NO3 levels was detected in the CRF-C group, the CRF-A group was characterized by a marked and immediate increase of plasma ARG [5].
  • PAP increased FBF more than fourfold, but did not affect serum nitrite concentration (n = 11), whereas ARG significantly increased both FBF and nitrite [20].

Co-localisations of RERE

  • Moreover, when RERE is overexpressed, the distribution of endogenous DRPLA protein alters from the diffused to the speckled pattern in the nucleus so as to co-localize with RERE [7].

Other interactions of RERE

  • Hybridization screening resulted in the rapid and comprehensive identification of partial cDNAs of which a portion comprised two novel candidate genes, termed DNB1/ARPh and DNB5, which encode putative proteins of 1011 and 447 amino acids, respectively [13].
  • Overexpression of RERE recruits a fraction of the proapoptotic protein BAX to PODS: This observation correlates with RERE-induced apoptosis, which occurs in a caspase-dependent manner [2].
  • In contrast, ATN-2 displayed a bathochromic shift only with hemopexin [21].

Analytical, diagnostic and therapeutic context of RERE

  • Immunoprecipitation and in vitro binding assays demonstrate that the DRPLA and RERE proteins bind each other, for which one of the RE repeats has a primary role, and extended polyglutamine enhances the binding [7].
  • The aim of this study is to assess whether gender and body mass index (BMI) should be considered in developing thresholds to define GH deficiency, using GH responses to GHRH + arginine (ARG) stimulation and insulin tolerance test (ITT) [22].
  • We employed in situ hybridization and immunocytochemical staining to monitor gene expression, and 5-bromo-2-deoxyuridine (BrdU), primary liquid cultures, and clonogenic progenitor assays to correlate AChE-R and ARP with proliferation and differentiation of hematopoietic progenitors [16].
  • We found that Aph, Agl, and Atr1, but not Atr2 or Atr3, were significantly smaller in the OSA group than in the control group [23].
  • These results were compared with those obtained by studying the effects of OGTT, Li-He and ARG on the GH response to GHRH-29 (1.0 micro g/kg i.v. at 0 min) [24].


  1. Novel differential gene expression in human cirrhosis detected by suppression subtractive hybridization. Shackel, N.A., McGuinness, P.H., Abbott, C.A., Gorrell, M.D., McCaughan, G.W. Hepatology (2003) [Pubmed]
  2. Human RERE is localized to nuclear promyelocytic leukemia oncogenic domains and enhances apoptosis. Waerner, T., Gardellin, P., Pfizenmaier, K., Weith, A., Kraut, N. Cell Growth Differ. (2001) [Pubmed]
  3. Inhibition of the growth of human pancreatic cancer cells by the arginine antimetabolite L-canavanine. Swaffar, D.S., Ang, C.Y., Desai, P.B., Rosenthal, G.A. Cancer Res. (1994) [Pubmed]
  4. GHRH plus arginine in the diagnosis of acquired GH deficiency of childhood-onset. Maghnie, M., Cavigioli, F., Tinelli, C., Autelli, M., Aricò, M., Aimaretti, G., Ghigo, E. J. Clin. Endocrinol. Metab. (2002) [Pubmed]
  5. Randomized, double-blind, placebo-controlled study of arginine supplementation in chronic renal failure. De Nicola, L., Bellizzi, V., Minutolo, R., Andreucci, M., Capuano, A., Garibotto, G., Corso, G., Andreucci, V.E., Cianciaruso, B. Kidney Int. (1999) [Pubmed]
  6. New approach to the diagnosis of growth hormone deficiency in adults. Ghigo, E., Aimaretti, G., Gianotti, L., Bellone, J., Arvat, E., Camanni, F. Eur. J. Endocrinol. (1996) [Pubmed]
  7. Protein binding of a DRPLA family through arginine-glutamic acid dipeptide repeats is enhanced by extended polyglutamine. Yanagisawa, H., Bundo, M., Miyashita, T., Okamura-Oho, Y., Tadokoro, K., Tokunaga, K., Yamada, M. Hum. Mol. Genet. (2000) [Pubmed]
  8. A new ETV6/TEL partner gene, ARG (ABL-related gene or ABL2), identified in an AML-M3 cell line with a t(1;12)(q25;p13) translocation. Iijima, Y., Ito, T., Oikawa, T., Eguchi, M., Eguchi-Ishimae, M., Kamada, N., Kishi, K., Asano, S., Sakaki, Y., Sato, Y. Blood (2000) [Pubmed]
  9. Short-term fasting in obesity fails to restore the blunted GH responsiveness to GH-releasing hormone alone or combined with arginine. Procopio, M., Maccario, M., Grottoli, S., Oleandri, S.E., Boffano, G.M., Camanni, F., Ghigo, E. Clin. Endocrinol. (Oxf) (1995) [Pubmed]
  10. APOBEC-1 and AID are nucleo-cytoplasmic trafficking proteins but APOBEC3G cannot traffic. Bennett, R.P., Diner, E., Sowden, M.P., Lees, J.A., Wedekind, J.E., Smith, H.C. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  11. Beta-hydroxy-beta-methylbutyrate supplementation in critically ill trauma patients. Kuhls, D.A., Rathmacher, J.A., Musngi, M.D., Frisch, D.A., Nielson, J., Barber, A., MacIntyre, A.D., Coates, J.E., Fildes, J.J. The Journal of trauma (2007) [Pubmed]
  12. Plasma arginine correlations in trauma and sepsis. Chiarla, C., Giovannini, I., Siegel, J.H. Amino Acids (2006) [Pubmed]
  13. Identification and characterization of novel genes located at the t(1;15)(p36.2;q24) translocation breakpoint in the neuroblastoma cell line NGP. Amler, L.C., Bauer, A., Corvi, R., Dihlmann, S., Praml, C., Cavenee, W.K., Schwab, M., Hampton, G.M. Genomics (2000) [Pubmed]
  14. Alterations in Fc receptor function of macrophages from streptozotocin-induced diabetic rats. Abrass, C.K., Hori, M. J. Immunol. (1984) [Pubmed]
  15. Hydrolytic and nonenzymatic functions of acetylcholinesterase comodulate hemopoietic stress responses. Grisaru, D., Pick, M., Perry, C., Sklan, E.H., Almog, R., Goldberg, I., Naparstek, E., Lessing, J.B., Soreq, H., Deutsch, V. J. Immunol. (2006) [Pubmed]
  16. ARP, a peptide derived from the stress-associated acetylcholinesterase variant, has hematopoietic growth promoting activities. Grisaru, D., Deutsch, V., Shapira, M., Pick, M., Sternfeld, M., Melamed-Book, N., Kaufer, D., Galyam, N., Gait, M.J., Owen, D., Lessing, J.B., Eldor, A., Soreq, H. Mol. Med. (2001) [Pubmed]
  17. Plasma arginine and urinary nitrate and nitrite excretion in bronchopulmonary dysplasia. Heckmann, M., Kreuder, J., Riechers, K., Tsikas, D., Boedeker, R.H., Reiss, I., Gortner, L. Biol. Neonate (2004) [Pubmed]
  18. Catabolism of aggrecan in cartilage explants. Identification of a major cleavage site within the interglobular domain. Sandy, J.D., Neame, P.J., Boynton, R.E., Flannery, C.R. J. Biol. Chem. (1991) [Pubmed]
  19. Reliability of provocative tests to assess growth hormone secretory status. Study in 472 normally growing children. Ghigo, E., Bellone, J., Aimaretti, G., Bellone, S., Loche, S., Cappa, M., Bartolotta, E., Dammacco, F., Camanni, F. J. Clin. Endocrinol. Metab. (1996) [Pubmed]
  20. Serum nitrite sensitively reflects endothelial NO formation in human forearm vasculature: evidence for biochemical assessment of the endothelial L-arginine-NO pathway. Kelm, M., Preik-Steinhoff, H., Preik, M., Strauer, B.E. Cardiovasc. Res. (1999) [Pubmed]
  21. Comparison of nonmetal and metal hydrophilic photosensitizer, ATX-S10 (Na) and ATN-2, binding with human serum proteins using spectrophotometry. Yamaguchi, M., Tanabe, S., Nakajima, S., Takemura, T., Ogita, K., Kuwayama, H., Sakata, I., Miyaki, S., Suzuki, K., Namiki, H., Uzuka, Y., Sarashina, T. Photochem. Photobiol. (2004) [Pubmed]
  22. Influence of body mass index and gender on growth hormone (GH) responses to GH-releasing hormone plus arginine and insulin tolerance tests. Qu, X.D., Gaw Gonzalo, I.T., Al Sayed, M.Y., Cohan, P., Christenson, P.D., Swerdloff, R.S., Kelly, D.F., Wang, C. J. Clin. Endocrinol. Metab. (2005) [Pubmed]
  23. Glottic and cervical tracheal narrowing in patients with obstructive sleep apnea. Rubinstein, I., Bradley, T.D., Zamel, N., Hoffstein, V. J. Appl. Physiol. (1989) [Pubmed]
  24. Effects of glucose, free fatty acids or arginine load on the GH-releasing activity of ghrelin in humans. Broglio, F., Benso, A., Gottero, C., Prodam, F., Grottoli, S., Tassone, F., Maccario, M., Casanueva, F.F., Dieguez, C., Deghenghi, R., Ghigo, E., Arvat, E. Clin. Endocrinol. (Oxf) (2002) [Pubmed]
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