Alms1 is an ubiquitous protein that is essential for normal primary ciliary function. Although its specific regulation mechanism has not been fully discovered, studies indicate that absent of Alms1 protein will promote nutrient absorption and increase food intake. Alms1 gene dysfunction is responsible for Alström syndrome in human. Alström syndrome is an autosomal recessive disorder of childhood obesity, type 2 diabetes, dyslipidemia, low growth hormone levels, hypothyroidism, infertility, and frequent abnormalities of kidney, heart, and liver; the latter includes steatosis and cirrhosisblindness. Mice with Alms1 gene deletion are susceptible to insulin resistance, obesity and diabetes. Futhermore, they show transition of steatosis to severe fibrosing steatohepatitis by feeding special diet.