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Gene Review

PREPL  -  prolyl endopeptidase-like

Homo sapiens

Synonyms: KIAA0436, Prolyl endopeptidase-like, Prolylendopeptidase-like
 
 
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Disease relevance of PREPL

 

High impact information on PREPL

  • In 11 patients with a recessive congenital disorder, which we refer to as "the hypotonia-cystinuria syndrome," microdeletion of part of the SLC3A1 and PREPL genes on chromosome 2p21 was found [3].
  • Deletion of PREPL, a gene encoding a putative serine oligopeptidase, in patients with hypotonia-cystinuria syndrome [3].
  • PREPL is localized in the cytosol and shows homology with prolyl endopeptidase and oligopeptidase B [3].
  • Comparison of Prepl with other autoregulated replication protein gene promoters revealed similarities, but Prepl differs from some of these characterized promoters in not being regulated by the heat-shock RNA polymerase [4].
  • Primer extension analysis showed that Prepl is contained within five helically aligned 18 base pair repeats, or 18-mers of the previously defined minimal origin [4].
 

Biological context of PREPL

 

Associations of PREPL with chemical compounds

  • The PREPL (previously called KIAA0436) gene encodes a putative serine peptidase from the prolyl oligopeptidase family [1].
  • Unexpectedly, the PREPL A protein did not cleave peptide substrates containing a P1 basic residue, but did slowly hydrolyse an activated ester substrate, and reacted with diisopropyl fluorophosphate [1].
  • This paper presents a semi-analytical expression to calculate (L/rho)medair at depths in the range of 0 to 30 g/cm2 and for any energy in the range of 4-60 MeV, for water, polystyrene, and acrylic relative to air, and another expression to calculate Prepl for any cylindrical ionization chamber with an inner diameter in the range of 3 to 7 mm [6].
 

Other interactions of PREPL

  • An analysis of the uncertainty introduced in the TG-21 factors (L/rho, Pwall, Prepl) caused by the spread in the measured data and translated into the determination of the TPR ratio results in an insignificant error (< 0.3%) [7].
 

Analytical, diagnostic and therapeutic context of PREPL

  • Proposed semi-analytical formulae for the determination of (L/rho)medair and Prepl for electron beams as used in radiotherapy [6].

References

  1. The PREPL A protein, a new member of the prolyl oligopeptidase family, lacking catalytic activity. Szeltner, Z., Alshafee, I., Juhász, T., Parvari, R., Polgár, L. Cell. Mol. Life Sci. (2005) [Pubmed]
  2. A recessive contiguous gene deletion of chromosome 2p16 associated with cystinuria and a mitochondrial disease. Parvari, R., Brodyansky, I., Elpeleg, O., Moses, S., Landau, D., Hershkovitz, E. Am. J. Hum. Genet. (2001) [Pubmed]
  3. Deletion of PREPL, a gene encoding a putative serine oligopeptidase, in patients with hypotonia-cystinuria syndrome. Jaeken, J., Martens, K., Francois, I., Eyskens, F., Lecointre, C., Derua, R., Meulemans, S., Slootstra, J.W., Waelkens, E., de Zegher, F., Creemers, J.W., Matthijs, G. Am. J. Hum. Genet. (2006) [Pubmed]
  4. Co-operative autoregulation of a replication protein gene. Gammie, A.E., Crosa, J.H. Mol. Microbiol. (1991) [Pubmed]
  5. PREPL: a putative novel oligopeptidase propelled into the limelight. Martens, K., Derua, R., Meulemans, S., Waelkens, E., Jaeken, J., Matthijs, G., Creemers, J.W. Biol. Chem. (2006) [Pubmed]
  6. Proposed semi-analytical formulae for the determination of (L/rho)medair and Prepl for electron beams as used in radiotherapy. Strydom, W.J., Korf, G.J. Medical dosimetry : official journal of the American Association of Medical Dosimetrists. (1995) [Pubmed]
  7. An empirical relationship for determining photon beam quality in TG-21 from a ratio of percent depth doses. Followill, D.S., Tailor, R.C., Tello, V.M., Hanson, W.F. Medical physics. (1998) [Pubmed]
 
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