title: A review of ERGIC-53: its structure, functions, regulation and relations with diseases.
abstract: ERGIC-53 is a type I transmembrane protein. It includes an N-terminal signal sequence, a carbohydrate recognition domain, which is calcium-dependent and pH-sensitive, a stalk region, a transmembrane domain, and a short cytoplasmic domain; ERGIC-53 mainly acts as a receptor of a limited number of glycoprotein and transports them from ER to ERGIC and Golgi, meanwhile it has a secondary glycoprotein quality control function. Recent research has revealed that UPR, heat shock and VIPL may regulate the expression of ERGIC-53. F5F8D, and some ER storage diseases have relationship with ERGIC-53.
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Tianjin, PR China.
authors.forename: Yong Ci
authors.initials: YC
authors.lastname: Zhang
authors.forename: Yuan
authors.initials: Y
authors.lastname: Zhou
authors.forename: Chun Zheng
authors.initials: CZ
authors.lastname: Yang
authors.forename: D S
authors.initials: DS
authors.lastname: Xiong
mesh_terms: D025861:Blood Coagulation Disorders, Inherited / Q000235:genetics; D004721:Endoplasmic Reticulum / Q000378:metabolism; D005165:Factor V / Q000235:genetics; D005169:Factor VIII / Q000235:genetics; D005786:Gene Expression Regulation; D005808:Genes, Recessive; D006023:Glycoproteins / Q000378:metabolism; D006056:Golgi Apparatus / Q000378:metabolism; D037241:Mannose-Binding Lectins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D008565:Membrane Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D011485:Protein Binding; D021382:Protein Sorting Signals; D017434:Protein Structure, Tertiary; D021381:Protein Transport; D033921:Vesicular Transport Proteins / Q000378:metabolism
chemical_list: D006023:Glycoproteins; C084004:LMAN1 protein, human; C475146:MCFD2 protein, human; D037241:Mannose-Binding Lectins; D008565:Membrane Proteins; D021382:Protein Sorting Signals; D033921:Vesicular Transport Proteins; D005165:Factor V; D005169:Factor VIII