BAFF - ling autoantibodies
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چکیده
5006 The Journal of Clinical Investigation http://www.jci.org Volume 123 Number 12 December 2013 1998;91(12):4397–4418. 5. Ruggeri ZM, Pareti FI, Mannucci PM, Ciavarella N, Zimmerman TS. Heightened interaction between platelets and Factor VIII/von Willebrand factor in a new subtype of von Willebrand’s disease. N Engl J Med. 1980;302(19):1047–1051. 6. Ware J, et al. Identification of a point mutation in type IIB von Willebrand disease illustrating the regulation of von Willebrand factor affinity for the platelet membrane glycoprotein Ib-IX receptor. Proc Natl Acad Sci U S A. 1991;88(7):2946–2950. 7. Celikel R, Ruggeri ZM, Varughese KI. von Willebrand factor conformation and adhesive function is modulated by an internalized water molecule. Nat Struct Biol. 2000;7(10):881–884. 8. Casari C, et al. von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin αIIbβ3. J Clin Invest. 2013;123(12):5071–5081. 9. Murata M, Ware J, Ruggeri ZM. Site-directed mutagenesis of a soluble recombinant fragment of platelet glycoprotein Ibα demonstrating negatively charged residues involved in von Willebrand factor binding. J Biol Chem. 1991;266(23):15474–15480. 10. Othman M, Lopez JA, Ware J. Platelet-type von Willebrand disease update: the disease, the molecule and the animal model. Expert Rev Hematol. 2011;4(5):475–477. 11. Guerrero JA, et al. Visualizing the von Willebrand factor/glycoprotein Ib-IX axis with a platelettype von Willebrand disease mutation. Blood. 2009;114(27):5541–5546. 12. Huizinga EG, et al. Structures of glycoprotein Ibα and its complex with von Willebrand factor A1 domain. Science. 2002;297(5584):1176–1179. important data to explain molecular consequences for a gain-of-function mutation in vWF many years after the recognition of type 2B as a distinct subtype of vWD.
منابع مشابه
Increased serum levels of B cell activating factor (BAFF) in subsets of patients with idiopathic inflammatory myopathies.
OBJECTIVE To investigate serum levels of B cell activating factor (BAFF) in patients with myositis and correlate these to autoantibody profile, clinical phenotype and treatment. METHODS BAFF levels in sera from 49 patients with dermatomyositis, 44 with polymyositis, 6 with inclusion body myositis and 30 matched controls were measured by ELISA. Specific autoantibodies were detected by line blo...
متن کاملThe Expression of BAFF Is Controlled by IRF Transcription Factors.
Patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) are typically characterized by the presence of autoantibodies and an IFN-signature. The strength of the IFN-signature positively correlates with disease severity, suggesting that type I IFNs are active players in these diseases. BAFF is a cytokine critical for development and proper selection of B cells, and t...
متن کاملBAFF and MyD88 signals promote a lupuslike disease independent of T cells
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the production of autoantibodies. However, the underlying cause of disease appears to relate to defects in T cell tolerance or T cell help to B cells. Transgenic (Tg) mice overexpressing the cytokine B cell-activating factor of the tumor necrosis factor family (BAFF) develop an autoimmune disorder similar to SL...
متن کاملIn vivo amelioration of endogenous antitumor autoantibodies via low-dose P4N through the LTA4H/activin A/BAFF pathway.
Cancer progression is associated with the development of antitumor autoantibodies in patients' sera. Although passive treatment with antitumor antibodies has exhibited remarkable therapeutic efficacy, inhibitory effects on tumor progression by endogenous antitumor autoantibodies (EAAs) have been limited. In this study, we show that P4N, a derivative of the plant lignan nordihydroguaiaretic acid...
متن کاملExpression of BAFF receptors in muscle tissue of myositis patients with anti-Jo-1 or anti-Ro52/anti-Ro60 autoantibodies
INTRODUCTION Anti-Jo-1 and anti-Ro52 autoantibodies are common in patients with myositis, but the mechanisms behind their production are not known. Survival of autoantibody-producing cells is dependent on B-cell-activating factor of the tumour necrosis factor family (BAFF). BAFF levels are elevated in serum of anti-Jo-1-positive myositis patients and are influenced by type-I interferon (IFN). I...
متن کاملdeltaBAFF, a splice isoform of BAFF, opposes full-length BAFF activity in vivo in transgenic mouse models.
DeltaBAFF is a novel splicing isoform of the regulator B cell-activating factor (BAFF, BLyS), a TNF family protein with powerful immunoregulatory effects. Overexpression of BAFF leads to excessive B cell accumulation, activation, autoantibodies, and lupus-like disease, whereas an absence of BAFF causes peripheral B cell immunodeficiency. Based on the ability of DeltaBAFF to multimerize with ful...
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