en Molecular Genetics & Genomics Molecular Genetics & Genomics مقاله مروری Review Article Coagulation factors belong to a family of plasma glycosylated proteins that should be activated for appropriate blood coagulation. Congenital deficiencies of these factors cause inheritable hemorrhagic diseases. Factor VII (FVII) deficiency is a rare bleeding disorder with variable clinical symptoms. Various mutations have been identified throughout the <em>F7</em> gene and can affect all the protein domains. The results of previous experiments have partly revealed the correlation between genotype and phenotype in patients with FVII deficiency<span dir="RTL">.</span> Nevertheless, each particular variant may affect the coagulative function of FVII, mainly via altering its expression level, extra-cellular secretion, tissue factor binding affinity, or proteolytic activity. The pathogenicity of the variants and molecular mechanisms responsible for clinical symptoms in patients with FVII deficiency should be characterized via <em>in silico</em> and <em>in vitro</em>, as well as<em> in vivo</em> functional studies. This review has highlighted the most important functional studies reported on <em>F7</em> gene variants, including relevant reports regarding Iranian FVII deficiency patients.&nbsp; Factor VII deficiency, in vitro techniques, mutation 165 174 http://ibj.pasteur.ac.ir/browse.php?a_code=A-10-1-774&slc_lang=en&sid=1 Shirin Shahbazi No Department of Medical Genetics, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran Reza Mahdian 0000-0003-1458-4767 Yes Molecular Medicine Department, Pasteur Institute of Iran, Tehran, Iran