重组Alexa Fluor® 555荧光Anti-LMAN1抗体[EPR6979]

补充信息

This supplementary information is collated from multiple sources and compiled automatically.

LMAN1 also known as ERGIC-53 or lectin mannose-binding 1 is a protein involved in the transport and quality control of glycoproteins. It has a mass of approximately 53 kDa. LMAN1 primarily expresses in the endoplasmic reticulum-Golgi intermediate compartment (ERGIC) and is important for efficient cargo sorting and proper folding. It functions by binding to specific carbohydrate moieties on glycoproteins aiding their transport between the ER and Golgi apparatus.

Biological function summary

LMAN1 functions significantly within a protein complex known as the LMAN1-MCFD2 complex. It serves an essential role as a cargo receptor for glycoproteins particularly those with particular mannose-rich domains influencing their secretion and stability. This protein complex is integral in maintaining protein homeostasis and cellular physiology by ensuring the correct sorting and transport of glycoproteins from the ER to the Golgi.

Pathways

LMAN1 significantly participates in the secretory pathway and quality control mechanisms in the cell. It interacts with proteins such as MCFD2 to mediate the transport of specific glycoproteins like α1-antitrypsin through the secretory pathway. This pathway is important for the secretion of a variety of proteins and disruptions can lead to disorders associated with improper protein folding and trafficking.

Defects in LMAN1 can lead to bleeding disorders such as combined coagulation factor V and VIII deficiency. The protein's dysfunction impairs the transport of coagulation factors disrupting their secretion. Additionally proteins like MCFD2 work closely with LMAN1 in this context as mutations in either protein can cause a similar deficiency highlighting the significant role of LMAN1 in maintaining normal hemostatic processes.