A well-established mouse model to study cLINCL was generated and characterized bytargeting the mouse gene Tpp1 in the Lobel laboratory (Sleat et al., 2004),resulting in loss of detectable TPP1 activity, progressive neurological phenotypesincluding ataxia, and increased motor deficiency. Here, TPP1 is linked to hyperinsulinemic hypoglycemia, familial, 4.