NEUROD1 (Neuronal Differentiation 1, also known as BETA2) is a Protein Coding gene. NEUROD1 encodes a transcription factor expressed in the endocrine pancreas and involved in beta-cell development, function, and mechanisms of apoptosis. The protein is a tissue-specific basic helix loop helix (bHLH) protein involved in the development and maintenance of the endocrine pancreas and neuronal elements. It forms heterodimers with other bHLH proteins and activates transcription of genes that contain a specific DNA sequence known as the E-box. NEUROD1 is expressed in both developing and mature beta-cells. Loss of NEUROD1 causes ataxia, cerebellar hypoplasia, sensorineural deafness, and severe retinal dystrophy in mice. Diseases associated with NEUROD1 include Maturity-Onset Diabetes Of The Young, Type 6 and Diabetes Mellitus, Noninsulin-Dependent.