<prompt> Which of the following is not true regarding Ataxia telangiectasia?Mutations in 11q gene is implicatedFollows autosomal dominant mode of inheritanceHumoral and cellular immunodeficiencyLinked to adenocarcinomas  <response>: The mode of inheritance in Ataxia telangiectasia is autosomal recessive Ataxia Telangiectasia Gene involved: Mutation in the ATM gene mapped to the long arm of chromosome 1111q22-23 Defective DNA repair, increased sensitivity to ionizing radiation Hypoplastic thymus Clinical Features : Progressive cerebellar ataxia Oculocutaneous telangiectasias Chronic sinopulmonary disease High incidence of malignancy: adenocarcinomas and lymphoreticular tumorslymphoma, leukemia, and Hodgkin disease Humoral and cellular immunodeficiency Raised alpha fetoprotein levels Ref: Nelson textbook of pediatrics 21st edition Pgno: 1117 