is a congenital, often familial, hemorrhagic disorder which was distinguished from classical hemophilia (AHG deficiency, factor VIII deficiency, hemophilia A) in 1952.13 Like the latter, it is usually inherited as a sex-linked recessive trait: only the male hemizygote for the abnormal gene is clinically affected, and the female heterozygote or carrier is asymptomatic. From a eugenic standpoint ...

Immunoassays with a monoclonal antibody (A-1) detect a prevalent dimorphism in plasma coagulation factor IX. The antibody was shown to react with a dimorphic segment of the normal factor IX sequence as follows. First, A-1 bound to isolated activation peptide (residues 146 through 180) prepared from activated factor IX from a normal plasma pool. Second, binding of recombinant factor IXs with A-1...

Thromb Haemost 2008; 99: 799–800 Developing effective cell-based therapies to treat severe haemophilia B remains attractive for several reasons. Ondemand and prophylactic factor IX replacement using plasma-derived or recombinant factor IX are both safe and effective (1). However, the cost of either replacement product for lifelong therapy is a significant barrier for patients with limited acces...

Background: Leukemia inhibitor factor (LIF) is a very important pleiotropic cytokine which belongs to interleukin-6 (IL-6) family. LIF exerts multiple effects on different types of cells and tissues with numerous regulatory effects in vivo and in vitro. It is a lymphoid factor, which performs a number of activities including cholinergic neuron differentia‌tion, contro...

is a congenital, often familial, hemorrhagic disorder which was distinguished from classical hemophilia (AHG deficiency, factor VIII deficiency, hemophilia A) in 1952.13 Like the latter, it is usually inherited as a sex-linked recessive trait: only the male hemizygote for the abnormal gene is clinically affected, and the female heterozygote or carrier is asymptomatic. From a eugenic standpoint ...

Previous genetic studies indicated intersex (ix) functions only in females and that it acts near the end of the sex determination hierarchy to control somatic sexual differentiation in Drosophila melanogaster. We have cloned ix and characterized its function genetically, molecularly and biochemically. The ix pre-mRNA is not spliced, and ix mRNA is produced in both sexes. The ix gene encodes a 1...

A partial gene product was identified in a pedigree with hemophilia B due to a partial deletion of the Factor IX gene (Chen, S.-H.,S. Yoshitake, P.F. Chance, G.L. Bray, A.R. Thompson, C.R. Scott, and K. Kurachi, 1985, J. Clin. Invest., 76:2161-2164). Levels of this mutant protein in plasma of affected family members studied ranged from 24 to 36 ng/ml (0.6-0.9 U/dl or percent of normal) by a sol...