Tyrosine-protein kinase BTK (BTK) is a tyrosine kinase that plays a crucial role in B-cell ontogeny. Defects in BTK are the cause of , Xenopus/Amphibian,-linked agammaglobulinemia (, Xenopus/Amphibian,LA) also known as , Xenopus/Amphibian,-linked agammaglobulinemia type 1 (AGM, Xenopus/Amphibian,1) or immunodeficiency type 1 (IMD1). , Xenopus/Amphibian,LA is a humoral immunodeficiency disease which results in developmental defects in the maturation pathway of B-cells. Affected boys have normal levels of pre-B-cells in their bone marrow but virtually no circulating mature B-lymphocytes. This results in a lack of immunoglobulins of all classes and leads to recurrent bacterial infections.