The mutation of TNNI3K, which expresses functional amino acid kinases with cardiac restricted expression patterns, does not directly affect the functional sites in the catalytic domain of ATP binding, but it has an effect on the assembly of proteins through certain signaling pathways such as MAPKs signaling pathway, mitochondrial signaling pathway, which ultimately leads to changes in a series of sarcometylum contraction regulatory proteins such as cardiac troponin I and cardiac actin, and ultimately promotes the occurrence of AT [71]. The gene discussed is TNNI3K; the disease is ataxia telangiectasia.