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Chemical Compound Review

proline     (2R)-pyrrolidine-2-carboxylic acid

Synonyms: D-Prolin, D-proline, R-Proline, D-Pro, D-PRO-OH, ...
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Disease relevance of proline

  • A proline-rich region conserved in all HIV-1 Gag polyproteins is required for cyclophilin A binding and incorporation [1].
  • Disruption of a single proline blocks the Gag-cyclophilin interaction in vitro, prevents cyclophilin A incorporation into virions, and inhibits HIV-1 replication [1].
  • Twelve percent of American patients with the blinding disease autosomal dominant retinitis pigmentosa (ADRP) carry a substitution of histidine for proline at codon 23 (P23H) in their rhodopsin gene, resulting in photoreceptor cell death from the synthesis of the abnormal gene product [2].
  • All of the three proline tRNA species, which read CCN codons in Salmonella typhimurium, have been sequenced and shown to contain m1G in position 37 [3].
  • An FKBP-rapamycin complex is concluded to be the toxic agent because (i) strains that lack FKBP proline rotamase, encoded by FPR1, were viable and fully resistant to rapamycin and (ii) FK506 antagonized rapamycin toxicity in vivo [4].

Psychiatry related information on proline


High impact information on proline


Chemical compound and disease context of proline


Biological context of proline


Anatomical context of proline

  • The inner ear Ush1c transcripts predicted several harmonin isoforms, some containing an additional coiled-coil domain and a proline- and serine-rich region [24].
  • Proline analogue removes fibroblasts from cultured mixed cell populations [25].
  • The FKBP and cyclophilin appear to be members of an emerging class of novel proteins that regulate T cell activation and other metabolic processes, perhaps by the recognition (and possibly the isomerization) of proline-containing epitopes in target proteins [26].
  • Mutation of this proline (Pro 87) in connexin 26 causes a reversal in the voltage-gating response when the mutant hemichannel is paired with wild-type connexin 26 in the Xenopus oocyte system [27].
  • Increased sensitivity of lymphocyte delta1-pyrroline-5-carboxylate reductase to inhibition by proline with transformation [28].

Associations of proline with other chemical compounds

  • Sequence analysis revealed a missense substitution of leucine (CTG) for proline (CCG) at residue 207 in exon 5 [29].
  • These results identify proline isomerization as a novel noncovalent histone modification that regulates transcription and provides evidence for crosstalk between histone lysine methylation and proline isomerization [12].
  • RESULTS: Four of the 121 obese subjects had a missense mutation in the gene for PPARgamma2 that resulted in the conversion of proline to glutamine at position 115, as compared with none of the 237 subjects of normal weight (P=0.01) [30].
  • Moreover, a histidine substitutes for a highly conserved proline at position 7 of the MSX2 homeodomain exclusively in affected members [31].
  • The protein has a novel two-domain structure with an amino-terminal domain homologous with erythropoietin and a carboxy-terminal domain rich in serine, threonine and proline residues and containing seven potential N-linked glycosylation sites [32].

Gene context of proline

  • We have isolated a novel gene from this region, AIRE (autoimmune regulator), which encodes a protein containing motifs suggestive of a transcription factor including two zinc-finger (PHD-finger) motifs, a proline-rich region and three LXXLL motifs [33].
  • The N-terminal proline-rich region of Oct-3, when fused to the DNA binding domain of c-Jun, functions as a transcriptional activating domain [34].
  • A newly identified proline-rich domain of Gab1 is responsible for the binding of this protein to the tyrosine-phosphorylated bidentate docking site in c-Met [35].
  • Alternatively, Pbs2p was activated by a mechanism that involves the binding of its amino terminal proline-rich motif to the Src homology 3 (SH3) domain of a putative transmembrane osmosensor Sho1p [36].
  • Far1-22p harbors a single amino acid change, from serine to proline at residue 87, which alters phosphorylation by Cdc28p-Cln2p in vitro [37].

Analytical, diagnostic and therapeutic context of proline

  • Quantitative sequence analyses suggest the existence of a common underlying structure in each TPR unit that consists of amphipathic alpha-helical regions punctuated by proline-induced turns [38].
  • Proline isomerism in staphylococcal nuclease characterized by NMR and site-directed mutagenesis [39].
  • Exactly 27 of the remaining fifth exons could encode a domain that is similar to those of the transplantation antigens in that it consists of a proline-rich connecting peptide, a transmembrane segment, and a cytoplasmic portion with membrane-anchoring basic residues [40].
  • Molecular cloning and expression of a high affinity L-proline transporter expressed in putative glutamatergic pathways of rat brain [41].
  • The mechanism of hydrolysis and absorption of a proline-containing tetrapeptide, Leu-Pro-Gly-Gly (10 mM) by rat intestine was examined in vivo by using jejunal perfusion methods [42].


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  2. Ribozyme rescue of photoreceptor cells in a transgenic rat model of autosomal dominant retinitis pigmentosa. Lewin, A.S., Drenser, K.A., Hauswirth, W.W., Nishikawa, S., Yasumura, D., Flannery, J.G., LaVail, M.M. Nat. Med. (1998) [Pubmed]
  3. Prevention of translational frameshifting by the modified nucleoside 1-methylguanosine. Björk, G.R., Wikström, P.M., Byström, A.S. Science (1989) [Pubmed]
  4. Targets for cell cycle arrest by the immunosuppressant rapamycin in yeast. Heitman, J., Movva, N.R., Hall, M.N. Science (1991) [Pubmed]
  5. Memory: proline induces retrograde amnesia in chicks. Cherkin, A., Eckardt, M.J., Gerbrandt, L.K. Science (1976) [Pubmed]
  6. Proline-directed phosphorylation and isomerization in mitotic regulation and in Alzheimer's Disease. Lu, K.P., Liou, Y.C., Vincent, I. Bioessays (2003) [Pubmed]
  7. Lidase treatment of spinal cord transected rats. Kowalski, T.F., Vahlsing, H.L., Feringa, E.R. Ann. Neurol. (1979) [Pubmed]
  8. L-Proline inhibition of glutamate release. Keller, E., Davis, J.L., Tachiki, K.H., Cummins, J.T., Baxter, C.F. J. Neurochem. (1981) [Pubmed]
  9. Decreased proline endopeptidase activity in the basal ganglia in Huntington's disease. Pittaway, K.M., Reynolds, G.P., Emson, P.C. J. Neurochem. (1984) [Pubmed]
  10. Proline hydroxylation and gene expression. Kaelin, W.G. Annu. Rev. Biochem. (2005) [Pubmed]
  11. Molecular biology of mammalian amino acid transporters. Malandro, M.S., Kilberg, M.S. Annu. Rev. Biochem. (1996) [Pubmed]
  12. Proline isomerization of histone h3 regulates lysine methylation and gene expression. Nelson, C.J., Santos-Rosa, H., Kouzarides, T. Cell (2006) [Pubmed]
  13. The FK506 binding protein Fpr3 counteracts protein phosphatase 1 to maintain meiotic recombination checkpoint activity. Hochwagen, A., Tham, W.H., Brar, G.A., Amon, A. Cell (2005) [Pubmed]
  14. The codon 72 polymorphic variants of p53 have markedly different apoptotic potential. Dumont, P., Leu, J.I., Della Pietra, A.C., George, D.L., Murphy, M. Nat. Genet. (2003) [Pubmed]
  15. The long terminal repeat sequences of a novel human endogenous retrovirus. O'Connell, C.D., Cohen, M. Science (1984) [Pubmed]
  16. Molecular biology of osmoregulation. Le Rudulier, D., Strom, A.R., Dandekar, A.M., Smith, L.T., Valentine, R.C. Science (1984) [Pubmed]
  17. Screening of germline mutations in the CDKN2A and CDKN2B genes in Swedish families with hereditary cutaneous melanoma. Platz, A., Hansson, J., Månsson-Brahme, E., Lagerlof, B., Linder, S., Lundqvist, E., Sevigny, P., Inganäs, M., Ringborg, U. J. Natl. Cancer Inst. (1997) [Pubmed]
  18. Decreased axonal transport of structural proteins in streptozotocin diabetic rats. Jakobsen, J., Sidenius, P. J. Clin. Invest. (1980) [Pubmed]
  19. Liver collagen synthesis in schistosomiasis mansoni. Dunn, M.A., Kamel, R., Kamel, I.A., Biempica, L., Kholy, A.E., Hait, P.K., Rojkind, M., Warren, K.S., Mahmoud, A.A. Gastroenterology (1979) [Pubmed]
  20. ATM associates with and phosphorylates p53: mapping the region of interaction. Khanna, K.K., Keating, K.E., Kozlov, S., Scott, S., Gatei, M., Hobson, K., Taya, Y., Gabrielli, B., Chan, D., Lees-Miller, S.P., Lavin, M.F. Nat. Genet. (1998) [Pubmed]
  21. Uncoating protein (hsc70) binds a conformationally labile domain of clathrin light chain LCa to stimulate ATP hydrolysis. DeLuca-Flaherty, C., McKay, D.B., Parham, P., Hill, B.L. Cell (1990) [Pubmed]
  22. The proline-rich transcriptional activator of CTF/NF-I is distinct from the replication and DNA binding domain. Mermod, N., O'Neill, E.A., Kelly, T.J., Tjian, R. Cell (1989) [Pubmed]
  23. Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus. Call, K.M., Glaser, T., Ito, C.Y., Buckler, A.J., Pelletier, J., Haber, D.A., Rose, E.A., Kral, A., Yeger, H., Lewis, W.H. Cell (1990) [Pubmed]
  24. A defect in harmonin, a PDZ domain-containing protein expressed in the inner ear sensory hair cells, underlies Usher syndrome type 1C. Verpy, E., Leibovici, M., Zwaenepoel, I., Liu, X.Z., Gal, A., Salem, N., Mansour, A., Blanchard, S., Kobayashi, I., Keats, B.J., Slim, R., Petit, C. Nat. Genet. (2000) [Pubmed]
  25. Proline analogue removes fibroblasts from cultured mixed cell populations. Kao, W.W., Prockop, D.J. Nature (1977) [Pubmed]
  26. A receptor for the immunosuppressant FK506 is a cis-trans peptidyl-prolyl isomerase. Harding, M.W., Galat, A., Uehling, D.E., Schreiber, S.L. Nature (1989) [Pubmed]
  27. Identification of a proline residue as a transduction element involved in voltage gating of gap junctions. Suchyna, T.M., Xu, L.X., Gao, F., Fourtner, C.R., Nicholson, B.J. Nature (1993) [Pubmed]
  28. Increased sensitivity of lymphocyte delta1-pyrroline-5-carboxylate reductase to inhibition by proline with transformation. Valle, D., Blaese, R.M., Phang, J.M. Nature (1975) [Pubmed]
  29. A mutation in the human lipoprotein lipase gene as the most common cause of familial chylomicronemia in French Canadians. Ma, Y., Henderson, H.E., Murthy, V., Roederer, G., Monsalve, M.V., Clarke, L.A., Normand, T., Julien, P., Gagné, C., Lambert, M. N. Engl. J. Med. (1991) [Pubmed]
  30. Obesity associated with a mutation in a genetic regulator of adipocyte differentiation. Ristow, M., Müller-Wieland, D., Pfeiffer, A., Krone, W., Kahn, C.R. N. Engl. J. Med. (1998) [Pubmed]
  31. A mutation in the homeodomain of the human MSX2 gene in a family affected with autosomal dominant craniosynostosis. Jabs, E.W., Müller, U., Li, X., Ma, L., Luo, W., Haworth, I.S., Klisak, I., Sparkes, R., Warman, M.L., Mulliken, J.B. Cell (1993) [Pubmed]
  32. Cloning and expression of murine thrombopoietin cDNA and stimulation of platelet production in vivo. Lok, S., Kaushansky, K., Holly, R.D., Kuijper, J.L., Lofton-Day, C.E., Oort, P.J., Grant, F.J., Heipel, M.D., Burkhead, S.K., Kramer, J.M. Nature (1994) [Pubmed]
  33. Positional cloning of the APECED gene. Nagamine, K., Peterson, P., Scott, H.S., Kudoh, J., Minoshima, S., Heino, M., Krohn, K.J., Lalioti, M.D., Mullis, P.E., Antonarakis, S.E., Kawasaki, K., Asakawa, S., Ito, F., Shimizu, N. Nat. Genet. (1997) [Pubmed]
  34. A novel octamer binding transcription factor is differentially expressed in mouse embryonic cells. Okamoto, K., Okazawa, H., Okuda, A., Sakai, M., Muramatsu, M., Hamada, H. Cell (1990) [Pubmed]
  35. Interaction between Gab1 and the c-Met receptor tyrosine kinase is responsible for epithelial morphogenesis. Weidner, K.M., Di Cesare, S., Sachs, M., Brinkmann, V., Behrens, J., Birchmeier, W. Nature (1996) [Pubmed]
  36. Activation of yeast PBS2 MAPKK by MAPKKKs or by binding of an SH3-containing osmosensor. Maeda, T., Takekawa, M., Saito, H. Science (1995) [Pubmed]
  37. Phosphorylation- and ubiquitin-dependent degradation of the cyclin-dependent kinase inhibitor Far1p in budding yeast. Henchoz, S., Chi, Y., Catarin, B., Herskowitz, I., Deshaies, R.J., Peter, M. Genes Dev. (1997) [Pubmed]
  38. A repeating amino acid motif in CDC23 defines a family of proteins and a new relationship among genes required for mitosis and RNA synthesis. Sikorski, R.S., Boguski, M.S., Goebl, M., Hieter, P. Cell (1990) [Pubmed]
  39. Proline isomerism in staphylococcal nuclease characterized by NMR and site-directed mutagenesis. Evans, P.A., Dobson, C.M., Kautz, R.A., Hatfull, G., Fox, R.O. Nature (1987) [Pubmed]
  40. Comparison of exon 5 sequences from 35 class I genes of the BALB/c mouse. Brorson, K.A., Hunt, S.W., Hunkapiller, T., Sun, Y.H., Cheroutre, H., Nickerson, D.A., Hood, L. J. Exp. Med. (1989) [Pubmed]
  41. Molecular cloning and expression of a high affinity L-proline transporter expressed in putative glutamatergic pathways of rat brain. Fremeau, R.T., Caron, M.G., Blakely, R.D. Neuron (1992) [Pubmed]
  42. Intestinal assimilation of a proline-containing tetrapeptide. Role of a brush border membrane postproline dipeptidyl aminopeptidase IV. Morita, A., Chung, Y.C., Freeman, H.J., Erickson, R.H., Sleisenger, M.H., Kim, Y.S. J. Clin. Invest. (1983) [Pubmed]
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