Apoliporotein H (ApoH, Beta-2 Glycoprotein I, Activated protein C-binding protein, Anticardiolipin cofactor or APC inhibitor) is a single chain glycoprotein consisting of 326 amino acid residues that can be divided into five short consensus repeat domains. ApoH has a molecular weight of about 50kDa and is synthesized by hepatocytes and circulates mainly in free form or associated with lipoproteins. ApoH is a major antigen for antiphospholipid antibodies present in patients with antiphospholipid syndrome (APS), which is related to venous and arterial thrombosis, fetal loss, and thrombocytopenia. Reports show that apoH may function in blood coagulation and in the clearance of apoptotic bodies from circulation. Serum concentrations of apoH have been found to be abnormal in patients with primary hyperlipidaemia. Significant correlations were demonstrated between plasma apoH concentrations and those of triglyceride and total cholesterol in some study groups. Regarding insulin resistance, serum apoH concentrations are increased in type 2 diabetes patients with microalbuminuria, suggesting a relationship with microvascular complications. ApoH was described as a major urinary protein in the urine of patients with renal tubular disorders such as Wilson´s disease, Dent´s syndrome, Fanconi´s syndrome and renal tubular acidosis.