Lymphoid hyperplasia is a common clinical finding. Patients develop recurrent bacterial sinopulmonary infections but do not develop opportunistic infections. UNG removes uracil residues generated by AID to help create DNA breaks that allow for class-switch recombination. UNG Deficiency (HIGM 5): UNG deficiency causes a B cell intrinsic form of HIGM that does not result in impaired function of T cells, monocytes, or dendritic cells. The switched memory B cell (CD27+IgD-IgM-) population is markedly reduced.
Patients have low IgG and IgA but normal or elevated IgM and poor specific IgG antibody responses. Lymphoid hyperplasia and autoimmunity (thrombocytopenia, hemolytic anemia, autoimmune hepatitis) are common clinical findings. Deamination of cytidine to uracil on single-stranded DNA by AID followed by the removal of uracil residues by UNG leads to the introduction of DNA breaks and initiation of class-switch recombination. AID is a protein expressed only in activated B cells. The impaired IL-12/IFN- signaling pathway is likely to be responsible for the phenotype of opportunistic infections.ĪID Deficiency (HIGM 2): AID deficiency causes a B cell intrinsic form of AR HIGM that does not result in impaired function of T cells, monocytes, or dendritic cells. The switched memory B cell population is markedly reduced. As a result, patients have low IgG and IgA but normal or elevated IgM and poor specific IgG antibody responses. The CD40-CD40L interaction is responsible for inducing antibody class-switching (from IgM to IgG, IgA, and IgE) and generating memory B cells. Patients are at risk for sinopulmonary infections with encapsulated bacteria as well as opportunistic infections (Pneumocystis and Cryptosporidium). CD40 deficiency is a combined B cell and T cell immunodeficiency while AID and UNG deficiency are B cell intrinsic immunodeficiencies.ĬD40 Deficiency (HIGM 3): CD40 deficiency causes an AR form of HIGM that is clinically identical to CD40 ligand deficiency. A fourth type of AR HIGM (HIGM 4) has been identified but the molecular cause has not been identified. Three types of autosomal recessive (AR) HIGM syndrome have been identified: CD40 deficiency, AID deficiency, and UNG deficiency. Both X-linked and autosomal recessive forms of disease have been described.Ģ. Hyper IgM syndrome (HIGM) is an antibody deficiency syndrome characterized by impaired class-switch recombination (which allows antibody isotype production to change from IgM to IgG, IgA, or IgE).
Literally bring in Thistleblade or Archspear and she will be melted down at an instant. She will draw Sev Gaus (her weapon), before firing either.One person per server, and you have one life. Sylvar Croati's badge description, Demon Slayer Earning this badge is quite the achievement. Every drop of blood spilled by Sylvar's disgusting weapon is yet another shame for her family to bear. Sylvar is the fifth child of a supreme demon lord and one of Yenti's grand god-generals who served during the first divine conflict. To many innocent towns along the rift, the sight of a strange drunken figure approaching in a bizarre swaying manner is a most assured symbol of their demise, and the demise of their friends and family. Since the third divine war Sylvar has been wandering both Earth and Divinia in a frenzied bout of battle-induced insanity. Her blood is liquid hellfire and her bones are high-density solid diamond. Defeat Sylvar Croati, a divine archdemon enhanced with bio-tech to have a hellfire core instead of a heart in order to become the perfect killing machine.
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