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Compound heterozygous AIRE-1 mutations causing autoimmune: Discussion

Our studies of a patient with APS1 have identified two AIRE-1 mutations, which consisted of a maternally inherited nonsense mutation (Arg257Stop), and a paternally inherited 13 bp deletional frameshift.The AIRE-1 gene encodes a 545 amino acid protein, that contains:2 plant … Continue reading →

Compound heterozygous AIRE-1 mutations causing autoimmune: Methods

Venous blood was obtained after informed consent, from the proband and his parents, using guidelines approved by the local ethical committee, and used to extract leukocyte DNA. Fourteen pairs of gene-specific oligonucleotide primers were employed for the PCR amplification of … Continue reading →

Compound heterozygous AIRE-1 mutations causing autoimmune

Presentation of clinical case The patient is at present a 9 year old boy who is the second child of non-consanguineous parents who are of British and Romany origin. He was born at full-term, by a normal delivery after an … Continue reading →

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