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zinc metallopeptidase (STE24 homolog, S. cerevisiae)
Symbol ZMPSTE24
Entrez 10269
HUGO 12877
OMIM 606480
RefSeq NM_005857
UniProt O75844
Other data
EC number
Locus Chr. 1 p34

ZMPSTE24 is a human gene.[1][2] The protein encoded by this gene is a metallopeptidase. It is involved in the processing of lamin A.[3] Defects in the ZMPSTE24 gene lead to similar laminopathies as defects in lamin A, because the latter is a substrate for the former.[4] In humans, a mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder[5]. Failure to correctly process prelamin A leads to deficient ability to repair DNA double-strand breaks[6][7].

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  1. ^ Tam A, Nouvet FJ, Fujimura-Kamada K, Slunt H, Sisodia SS, Michaelis S (August 1998). "Dual roles for Ste24p in yeast a-factor maturation: NH2-terminal proteolysis and COOH-terminal CAAX processing". J. Cell Biol. 142 (3): 635–49. doi:10.1083/jcb.142.3.635. PMC 2148179Freely accessible. PMID 9700155. 
  2. ^ Freije JM, Blay P, Pendás AM, Cadiñanos J, Crespo P, López-Otín C (June 1999). "Identification and chromosomal location of two human genes encoding enzymes potentially involved in proteolytic maturation of farnesylated proteins". Genomics. 58 (3): 270–80. doi:10.1006/geno.1999.5834. PMID 10373325. 
  3. ^ Young SG, Fong LG, Michaelis S (December 2005). "Prelamin A, Zmpste24, misshapen cell nuclei, and progeria--new evidence suggesting that protein farnesylation could be important for disease pathogenesis". J. Lipid Res. 46 (12): 2531–58. doi:10.1194/jlr.R500011-JLR200. PMID 16207929. 
  4. ^ Varela I, Cadiñanos J, Pendás AM, Gutiérrez-Fernández A, Folgueras AR, Sánchez LM, Zhou Z, Rodríguez FJ, Stewart CL, Vega JA, Tryggvason K, Freije JM, López-Otín C (September 2005). "Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation". Nature. 437 (7058): 564–8. doi:10.1038/nature04019. PMID 16079796. 
  5. ^ Wang Y, Lichter-Konecki U, Anyane-Yeboa K, Shaw JE, Lu JT, Östlund C, Shin JY, Clark LN, Gundersen GG, Nagy PL, Worman HJ (2016). "A mutation abolishing the ZMPSTE24 cleavage site in prelamin A causes a progeroid disorder". J. Cell. Sci. 129 (10): 1975–80. doi:10.1242/jcs.187302. PMID 27034136. 
  6. ^ Redwood AB, Perkins SM, Vanderwaal RP, Feng Z, Biehl KJ, Gonzalez-Suarez I, Morgado-Palacin L, Shi W, Sage J, Roti-Roti JL, Stewart CL, Zhang J, Gonzalo S (2011). "A dual role for A-type lamins in DNA double-strand break repair". Cell Cycle. 10 (15): 2549–60. doi:10.4161/cc.10.15.16531. PMC 3180193Freely accessible. PMID 21701264. 
  7. ^ Gonzalo S, Kreienkamp R (2015). "DNA repair defects and genome instability in Hutchinson-Gilford Progeria Syndrome". Curr. Opin. Cell Biol. 34: 75–83. doi:10.1016/ PMC 4522337Freely accessible. PMID 26079711. 

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