Eszter Kozák, Bence Szikora, Attila Iliás, Péter K Jani, Zoltán Hegyi, Zsolt Matula, Dóra Dedinszki, Natália Tőkési, Krisztina Fülöp, Viola Pomozi, György Várady, Éva Bakos, Gabor E Tusnády, Imre Kacskovics, Andras Váradi
Abstract
Mutations in the ABCC6 gene result in calcification diseases such as pseudoxanthoma elasticum or Generalized Arterial Calcification of Infancy. Generation of antibodies recognizing an extracellular (EC) epitope of ABCC6 has been hampered by the short EC segments of the protein. To overcome this limitation, we immunized bovine FcRn transgenic mice exhibiting an augmented humoral immune response with Human Embryonic Kidney 293 cells cells expressing human ABCC6 (hABCC6). We obtained a monoclonal antibody recognizing an EC epitope of hABCC6 that we named mEChC6. Limited proteolysis revealed that the epitope is within a loop in the N-terminal half of ABCC6 and probably spans amino acids 338-347. mEChC6 recognizes hABCC6 in the liver of hABCC6 transgenic mice, verifying both specificity and EC binding to intact hepatocytes.
https://pubmed.ncbi.nlm.nih.gov
Kozák et al.
FEBS Lett. 2021 Mar;595(6):789-798. doi: 10.1002/1873-3468.13991.
