More recently, glucose metabolism and pancreatic abnormalities were uncovered in an intermediate SMA mouse model and Type 1 SMA patients such as a dramatic predominance of glucagon-producing α cells at the expense of insulin-producing β cells within pancreatic islets, fasting hyperglycemia, hyperglucagonemia, glucose resistance and increased hepatic insulin sensitivity (Bowerman et al., 2012c). Here, INS is linked to spinal muscular atrophy, type 1.