Gene Symbol: CHML
Description: CHM like, Rab escort protein 2
Alias: REP2, rab proteins geranylgeranyltransferase component A 2, choroideraemia-like protein, choroideremia-like (Rab escort protein 2), choroideremia-like protein
Species: human
Products:     CHML

Top Publications

  1. McQuaid M, Mereshchuk A, Dobson M. Insights into the DNA sequence elements required for partitioning and copy number control of the yeast 2-micron plasmid. Curr Genet. 2019;: pubmed publisher
    ..established, the partitioning system and the means by which the 2-micron plasmid partitioning proteins, Rep1 and Rep2, regulate plasmid copy number remain incompletely understood...
  2. Tian A, Lu M, Zhang F, Calderón Mantilla G, Petsalaki E, Tian X, et al. The pervasive effects of recombinant Fasciola gigantica Ras-related protein Rab10 on the functions of goat peripheral blood mononuclear cells. Parasit Vectors. 2018;11:579 pubmed publisher
    ..interactions between Rab proteins geranylgeranyltransferase component A 1 (CHM) and Rab proteins geranylgeranyltransferase component A 2 (CHML) and rFgRab10 protein...
  3. Mukherjee K, Futcher B, Leatherwood J. mmi1 and rep2 mRNAs are novel RNA targets of the Mei2 RNA-binding protein during early meiosis in Schizosaccharomyces pombe. Open Biol. 2018;8: pubmed publisher meiRNA, we found the mRNAs for mmi1 (which encodes Mmi1) and for the S-phase transcription factor rep2 There were also three other RNAs of uncertain relevance...
  4. Singh P, Khatri I, Jha A, Pretto C, Spindler K, Arumugaswami V, et al. Determination of system level alterations in host transcriptome due to Zika virus (ZIKV) Infection in retinal pigment epithelium. Sci Rep. 2018;8:11209 pubmed publisher
    ..Interestingly, the ZIKV infection signature revealed the downregulation of ALDH5A1 and CHML, genes implicated in neurological (cognitive impairment, expressive language deficit, and mild ataxia) and ..
  5. Zheleznyakova G, Nilsson E, Kiselev A, Maretina M, Tishchenko L, Fredriksson R, et al. Methylation levels of SLC23A2 and NCOR2 genes correlate with spinal muscular atrophy severity. PLoS ONE. 2015;10:e0121964 pubmed publisher
    ..CpG sites, designed as "targets", as well as the nearest CpG sites in regulatory regions of ARHGAP22, CDK2AP1, CHML, NCOR2, SLC23A2 and RPL9 in three groups of SMA patients...
  6. Rizvi S, Prajapati H, Ghosh S. The 2 micron plasmid: a selfish genetic element with an optimized survival strategy within Saccharomyces cerevisiae. Curr Genet. 2018;64:25-42 pubmed publisher
    ..It codes for four proteins (Rep1, Rep2, Raf1, and Flp) vital for its own survival and recruits several host factors (RSC2, Cohesin, Cse4, Kip1, Bik1, Bim1,..
  7. Prajapati H, Rizvi S, Rathore I, Ghosh S. Microtubule-associated proteins, Bik1 and Bim1, are required for faithful partitioning of the endogenous 2 micron plasmids in budding yeast. Mol Microbiol. 2017;103:1046-1064 pubmed publisher
    ..The partitioning system consists of the plasmid-borne proteins Rep1, Rep2 and a cis-acting locus STB that, along with several host factors, ensures efficient segregation of the plasmid...
  8. Wardal E, Kuch A, Gawryszewska I, Zabicka D, Hryniewicz W, Sadowy E. Diversity of plasmids and Tn1546-type transposons among VanA Enterococcus faecium in Poland. Eur J Clin Microbiol Infect Dis. 2017;36:313-328 pubmed publisher
    ..The vanA-determinant was typically located on plasmids, which most commonly carried rep2pRE25, rep17pRUM, rep18pEF418, rep1pIP501, ?-?-? and axe-txe genes...
  9. Bolzenius J, Cushman R, Perry G. Expression of Na(+)/H(+) exchanger isoforms 1, 2, 3, and 4 in bovine endometrium and the influence of uterine pH at time of fixed-time AI of pregnancy success. Anim Reprod Sci. 2016;171:98-107 pubmed publisher
    ..1), 72, 84, or 96h (Rep2) after CIDR removal, and total cellular RNA was extracted from all biopsies...

More Information


  1. Wang X, Wu Z, Xiang Q, Li Z, Zhang R, Chen J, et al. Characterization of the nuclear localization signals of duck circovirus replication proteins. Acta Virol. 2015;59:423-8 pubmed
    ..Based on the viral genotype, there are two categories of Rep proteins: Rep1 and Rep2. To characterize the nuclear localization signals (NLSs) conferring the nuclear localization of the Rep proteins, ..
  2. White J, Chiano M, Wigglesworth M, Geske R, Riley J, White N, et al. Identification of a novel asthma susceptibility gene on chromosome 1qter and its functional evaluation. Hum Mol Genet. 2008;17:1890-903 pubmed publisher
    ..Further screening using the pedigree-based association test (PBAT) identified polymorphisms in the OPN3 and CHML genes as being associated with asthma and atopic asthma after correcting for multiple comparisons...
  3. McQuaid M, Pinder J, Arumuggam N, Lacoste J, Chew J, Dobson M. The yeast 2-?m plasmid Raf protein contributes to plasmid inheritance by stabilizing the Rep1 and Rep2 partitioning proteins. Nucleic Acids Res. 2017;45:10518-10533 pubmed publisher
    ..Equal partitioning of the plasmid upon host cell division requires plasmid proteins Rep1 and Rep2 and the plasmid STB locus...
  4. McCauley J, Zuvich R, Bradford Y, Kenealy S, Schnetz Boutaud N, Gregory S, et al. Follow-up examination of linkage and association to chromosome 1q43 in multiple sclerosis. Genes Immun. 2009;10:624-30 pubmed publisher
    ..We found preliminary evidence to suggest that common variation within the RGS7 locus may be involved in disease susceptibility. ..
  5. Cremers F, Molloy C, van de Pol D, van den Hurk J, Bach I, Geurts van Kessel A, et al. An autosomal homologue of the choroideremia gene colocalizes with the Usher syndrome type II locus on the distal part of chromosome 1q. Hum Mol Genet. 1992;1:71-5 pubmed
    ..The colocalization of hCHML and Usher syndrome type II, as well as the clinical similarities between choroideremia and Usher syndrome type II, make hCHML a candidate gene for this disorder...
  6. Schluter A, Szczepanowski R, Kurz N, Schneiker S, Krahn I, Puhler A. Erythromycin resistance-conferring plasmid pRSB105, isolated from a sewage treatment plant, harbors a new macrolide resistance determinant, an integron-containing Tn402-like element, and a large region of unknown function. Appl Environ Microbiol. 2007;73:1952-60 pubmed
    ..Accordingly, pRSB101 and pRSB105 are sister plasmids belonging to a new plasmid family. The second replicon (Rep2) of pRSB105 was classified as a member of the IncP-6 group...
  7. Rak A, Pylypenko O, Niculae A, Pyatkov K, Goody R, Alexandrov K. Structure of the Rab7:REP-1 complex: insights into the mechanism of Rab prenylation and choroideremia disease. Cell. 2004;117:749-60 pubmed
  8. Pereira Leal J, Strom M, Godfrey R, Seabra M. Structural determinants of Rab and Rab Escort Protein interaction: Rab family motifs define a conserved binding surface. Biochem Biophys Res Commun. 2003;301:92-7 pubmed
    ..These results support the hypothesis that Rabs bind REP via conserved RabF motifs and provide a molecular explanation for the preferential recognition of the GDP-bound conformation of Rab by REP. ..
  9. Sidorovitch V, Niculae A, Kan N, Ceacareanu A, Alexandrov K. Expression of mammalian Rab Escort protein-1 and -2 in yeast Saccharomyces cerevisiae. Protein Expr Purif. 2002;26:50-8 pubmed
    ..Purified proteins were functionally active, as determined by a fluorescent Rab binding assay and in vitro prenylation. The reported procedure provides a reliable source of REP proteins for biochemical and structural studies. ..
  10. Kasper G, Taudien S, Staub E, Mennerich D, Rieder M, Hinzmann B, et al. Different structural organization of the encephalopsin gene in man and mouse. Gene. 2002;295:27-32 pubmed
    ..e. the number of genes within a certain cluster may differ between man and mouse. OPN3 provides an exception to this rule, since it is positionally uncoupled from other genes of the opsin family. ..
  11. Chan D, Lin J, Raffaniello R. Expression and localization of rab escort protein isoforms in parotid acinar cells from rat. J Cell Physiol. 2000;185:339-47 pubmed
    ..In addition, unlike other Rab proteins, cytosolic Rab3D appears to associate exclusively with REP, not rab-GDI in parotid acinar cells. ..
  12. Anant J, Desnoyers L, Machius M, Demeler B, Hansen J, Westover K, et al. Mechanism of Rab geranylgeranylation: formation of the catalytic ternary complex. Biochemistry. 1998;37:12559-68 pubmed
    ..Our data suggest a model whereby REP behaves kinetically as an essential activator of the reaction. ..
  13. Cremers F, Armstrong S, Seabra M, Brown M, Goldstein J. REP-2, a Rab escort protein encoded by the choroideremia-like gene. J Biol Chem. 1994;269:2111-7 pubmed
    ..In the retina, REP-2 may be only partially effective, leading eventually to retinal degeneration and blindness...
  14. von Bokhoven H, von Genderen C, Molloy C, van de Pol D, Cremers C, von Aarem A, et al. Mapping of the choroideremia-like (CHML) gene at 1q42-qter and mutation analysis in patients with Usher syndrome type II. Genomics. 1994;19:385-7 pubmed
    The human choroideremia-like (CHML) gene and a locus for Usher syndrome type 2 (USH2) were recently mapped to the 1q31-qter region employing physical mapping and genetic linkage studies, respectively...
  15. Seabra M, Ho Y, Anant J. Deficient geranylgeranylation of Ram/Rab27 in choroideremia. J Biol Chem. 1995;270:24420-7 pubmed
    ..These results raise the possibility that the retinal degeneration in choroideremia results from the deficient geranylgeranylation of Ram/Rab27 or a closely related protein...
  16. van Bokhoven H, van den Hurk J, Bogerd L, Philippe C, Gilgenkrantz S, de Jong P, et al. Cloning and characterization of the human choroideremia gene. Hum Mol Genet. 1994;3:1041-6 pubmed highly homologous to the mouse and rat CHM proteins, and, to a slightly lesser extent, to the human CHM-like (CHML) protein. The open reading frame (ORF) of the human CHM gene consists of 15 exons, spanning at least 150 kb of Xq21...