Endoplasmic reticulum-Golgi intermediate compartment 53 kDa protein (LMAN1)

Endoplasmic reticulum-Golgi intermediate compartment 53 kDa protein (LMAN1, also known as ERGIC-53) is a type I integral membrane mannose-specific lectin cycling between the endoplasmic reticulum (ER), the ER-Golgi intermediate compartment (ERGIC), and cis-Golgi. It functions as a cargo receptor for the ER-to-Golgi transport of selected glycoproteins, including blood clotting factors V and VIII, by forming a complex with MCFD2. Loss-of-function mutations result in combined deficiency of coagulation factors V and VIII, an autosomal recessive bleeding disorder. LMAN1 has also been implicated in colorectal tumorigenesis through early mutational inactivation. Its mechanism involves a calcium-dependent, pH-sensitive carbohydrate recognition domain, and it is not a direct target of any approved therapeutic drugs.

Other names

ERGIC-53Protein ERGIC-53Lectin, mannose-binding 1Intracellular mannose-specific lectin MR60MR60F5F8Dgp58Endoplasmic reticulum-Golgi intermediate compartment protein 53ER-Golgi intermediate compartment 53 kDa proteinLectin mannose-binding 1

Not drug-targeted directly; mutations in LMAN1 disrupt ER-to-Golgi transport of clotting factors V and VIII, leading to combined deficiency (loss of function).