A review of ERGIC-53: its structure, functions, regulation and relations with diseases

Histol Histopathol. 2009 Sep;24(9):1193-204. doi: 10.14670/HH-24.1193.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Blood Coagulation Disorders, Inherited / genetics
  • Endoplasmic Reticulum / metabolism
  • Factor V / genetics
  • Factor VIII / genetics
  • Gene Expression Regulation
  • Genes, Recessive
  • Glycoproteins / metabolism
  • Golgi Apparatus / metabolism
  • Mannose-Binding Lectins / chemistry*
  • Mannose-Binding Lectins / genetics
  • Mannose-Binding Lectins / metabolism*
  • Membrane Proteins / chemistry*
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism*
  • Protein Binding
  • Protein Sorting Signals
  • Protein Structure, Tertiary
  • Protein Transport
  • Vesicular Transport Proteins / metabolism

Substances

  • Glycoproteins
  • LMAN1 protein, human
  • MCFD2 protein, human
  • Mannose-Binding Lectins
  • Membrane Proteins
  • Protein Sorting Signals
  • Vesicular Transport Proteins
  • Factor V
  • Factor VIII