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The recently detected cholesterol deficiency (CD) in Holstein cattle is associated with reduced cholesterol in calves and bulls and caused by a loss of function mutation of the APOB gene. We set out to investigate if this mutation influences also the metabolism and performance of dairy cows. Twenty pairs of full sibling cows from same farms were investigated. Each pair contained a heterozygous carrier (CDC) and a non-carrier (CDF) of the disease associated APOB mutation. Blood samples were taken at 3-4 weeks and 4-6 months in lactation to measure cholesterol metabolites. A paired t-test was used to compare both groups. Total and free cholesterol (TC), high density lipoproteins, low density lipoproteins, phospholipids and cholesterol esters, but not triacylglycerides and very low density lipoproteins were lower in CDC cows compared with matched CDF siblings at both time points (P< 0.05). Concentrations of …