Late endosomal/lysosomal adaptor and MAPK and MTOR activator 2
Late endosomal/lysosomal Mp1 interacting protein
Late endosomal/lysosomal Mp1-interacting protein
MAPKSP1 adaptor protein
Mitogen activated protein binding protein interacting protein
Mitogen-activated protein-binding protein-interacting protein
Ragulator complex protein LAMTOR2
Roadblock domain containing 3
Roadblock domain containing protein 3
Roadblock domain-containing protein 3
FunctionAs part of the Ragulator complex it is involved in amino acid sensing and activation of mTORC1, a signaling complex promoting cell growth in response to growth factors, energy levels, and amino acids. Activated by amino acids through a mechanism involving the lysosomal V-ATPase, the Ragulator functions as a guanine nucleotide exchange factor activating the small GTPases Rag. Activated Ragulator and Rag GTPases function as a scaffold recruiting mTORC1 to lysosomes where it is in turn activated. Adapter protein that enhances the efficiency of the MAP kinase cascade facilitating the activation of MAPK2.
Involvement in diseaseDefects in LAMTOR2 are the cause of immunodeficiency due to defect in MAPBP-interacting protein (ID-MAPBPIP) [MIM:610798]. This form of primary immunodeficiency syndrome includes congenital neutropenia, partial albinism, short stature and B-cell and cytotoxic T-cell deficiency.