Familial hypercatabolic hypoproteinemia caused by deficiency of the neonatal Fc receptor, FcRn, due to a mutant beta2-microglobulin gene.

@article{Wani2006FamilialHH,
  title={Familial hypercatabolic hypoproteinemia caused by deficiency of the neonatal Fc receptor, FcRn, due to a mutant beta2-microglobulin gene.},
  author={Manzoor A. Wani and Lynn D Haynes and Jonghan Kim and Cassaundra Bronson and Chaity Chaudhury and Sudhasri Mohanty and Thomas A. Waldmann and J. Michael Robinson and Clark Anderson},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2006},
  volume={103 13},
  pages={5084-9}
}
Two siblings, products of a consanguineous marriage, were markedly deficient in both albumin and IgG because of rapid degradation of these proteins, suggesting a lack of the neonatal Fc receptor, FcRn. FcRn is a heterodimeric receptor composed of a nonclassical MHC class I alpha-chain and beta(2)-microglobulin (beta(2)m) that binds two ligands, IgG and albumin, and extends the catabolic half-lives of both. Eight relatives of the siblings were moderately IgG-deficient. From sera archived for 35… CONTINUE READING
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