The endoplasmic reticulum (ER) is one of the most critical infra-cellular structures with a wide variety of functions and mutations in coding genes neutrophil elastase (ELANE), Glucose-6-phosphatase catalytic 3 (G6PC3), and Jagunal homolog 1 (JAGN1) are associated with SCN (Table 1) [4–6]. The gene discussed is ELANE; the disease is severe congenital neutropenia.