Protein Z polymorphisms associated with vaso-occlusive crisis in young sickle cell disease patients

Annals of Hematology
Najat MahdiWassim Y Almawi

Abstract

We investigated the association of protein Z (PZ) promoter (rs3024718, rs3024719, and rs3024731) and intron (rs3024735; G79A) SNPs with sickle cell disease (SCD) vaso-occlusive crisis (VOC). Study subjects included 239 SCD patients with VOC and 138 pain-free SCD control patients. PZ genotyping was done by allelic discrimination (real-time PCR) assays. The minor allele frequency of rs3024718 (P=0.03), rs3024719 (P=0.02), rs3024731 (P<0.001), and rs3024735 (P<0.001) were higher in VOC patients than control SCD patients. Significant differences in the distribution of rs3024731 (P=0.028) and rs3024735 (P=0.045) genotypes were seen between VOC and steady-state SCD patients. This association remained significant after adjusting for gender, HbS, and HbF. Four-locus (rs3024718/rs3024719/rs3024731/rs3024735) PZ haplotypes analysis demonstrated increased frequency of GAAA (P=0.024), AGAA (P=0.011), and GGTG (P=0.002), and reduced frequency of AGTG haplotype (P=0.001) in VOC than in steady-state control patients, thereby conferring disease susceptibility and protective nature to these haplotypes, respectively. Of these, only AGTG (P(c)=0.001) and GGTG (P(c)=0.018) remained significant after applying the Bonferroni correction. In conclusio...Continue Reading

References

Jun 1, 2000·Proceedings of the National Academy of Sciences of the United States of America·Z F YinG J Broze
Sep 13, 2003·Stroke; a Journal of Cerebral Circulation·Andrew M McQuillanVanessa Cole
Jun 29, 2004·Journal of Thrombosis and Haemostasis : JTH·R SantacroceM Margaglione
Aug 24, 2004·Annals of Hematology·Emel GürkanFikri Başlamişli
Mar 1, 2005·Thrombosis and Haemostasis·Ali Al-ShanqeetiGeorge J Broze
Apr 21, 2005·Thrombosis and Haemostasis·Katharina C Quack LoetscherRoland Zimmermann
May 10, 2005·Stroke; a Journal of Cerebral Circulation·J StatonJ W Eikelboom
Jul 30, 2005·Journal of Thrombosis and Thrombolysis·Richard C Becker
Aug 30, 2006·Journal of Thrombosis and Haemostasis : JTH·R SantacroceM Margaglione
Mar 6, 2007·Thrombosis and Haemostasis·Mary J HeebAnnlia Paganini-Hill
Aug 4, 2007·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Juan Pedro KusanovicSonia S Hassan
Aug 19, 2007·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Offer ErezTinnakorn Chaiworapongsa
Nov 23, 2007·Thrombosis Research·Thomas SailerIngrid Pabinger
May 27, 2008·The Journal of Biological Chemistry·Alireza R RezaieLikui Yang
Oct 9, 2008·Thrombosis and Haemostasis·Marc Vasse
May 13, 2009·Journal of the American Academy of Nurse Practitioners·Ardie Pack-Mabien, Johnson Haynes
Jul 3, 2009·Thrombosis and Haemostasis·Véronique Le Cam-DuchezJean-Philippe Collet
Jul 11, 2009·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Offer ErezSonia S Hassan
Nov 3, 2010·Annals of the New York Academy of Sciences·Vijay G SankaranJoel N Hirschhorn
Dec 7, 2010·Lancet·David C ReesMark T Gladwin
Apr 2, 2011·Seminars in Thrombosis and Hemostasis·Lucia De FranceschiOliviero Olivieri
Sep 14, 2011·Hemoglobin·Samir K Ballas
Sep 29, 2011·Clinical and Applied Thrombosis/hemostasis : Official Journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis·M H QariS A Mousa

❮ Previous
Next ❯

Citations

Apr 17, 2014·European Journal of Haematology·Deepika S DarbariDaniel J Clauw
May 27, 2017·Journal of Clinical Laboratory Analysis·Mervat M KhorshiedMona K El-Ghamrawy

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.