IGF2R

This gene encodes a receptor for both insulin-like growth factor 2 and mannose 6-phosphate. The binding sites for each ligand are located on different segments of the protein. This receptor has various functions, including in the intracellular trafficking of lysosomal enzymes, the activation of transforming growth factor beta, and the degradation of insulin-like growth factor 2. Mutation or loss of heterozygosity of this gene has been association with risk of hepatocellular carcinoma. The orthologous mouse gene is imprinted and shows exclusive expression from the maternal allele; however, imprinting of the human gene may be polymorphic, as only a minority of individuals showed biased expression from the maternal allele (PMID:8267611). [provided by RefSeq, Nov 2015]

Insulin Like Growth Factor 2 Receptor

Mediates the transport of phosphorylated lysosomal enzymes from the Golgi complex and the cell surface to lysosomes (PubMed:2963003, PubMed:18817523).

Lysosomal enzymes bearing phosphomannosyl residues bind specifically to mannose-6-phosphate receptors in the Golgi apparatus and the resulting receptor-ligand complex is transported to an acidic prelysosomal compartment where the low pH mediates the dissociation of the complex (PubMed:2963003, PubMed:18817523).

The receptor is then recycled back to the Golgi for another round of trafficking through its binding to the retromer (PubMed:18817523).

This receptor also binds IGF2 (PubMed:18046459).

Acts as a positive regulator of T-cell coactivation by binding DPP4 (PubMed:10900005).

Animal organ regeneration Source: Ensembl
G protein-coupled receptor signaling pathway Source: Ensembl
Liver development Source: Ensembl
Lysosomal transport Source: InterPro
Positive regulation by host of viral process Source: ARUK-UCL
Positive regulation of apoptotic process Source: Ensembl
Post-embryonic development Source: Ensembl
Receptor-mediated endocytosis Source: ProtInc
Response to retinoic acid Source: Ensembl
Response to tetrachloromethane Source: Ensembl
Signal transduction Source: ProtInc
Spermatogenesis Source: Ensembl

Endosome membrane; Golgi apparatus membrane. Mainly localized in the Golgi at steady state and not detectable in lysosome (PubMed:18817523). Colocalized with DPP4 in internalized cytoplasmic vesicles adjacent to the cell surface (PubMed:10900005).

Lumenal: 41-2304
Helical: 2305-2327
Cytoplasmic: 2328-2491

Palmitoylated (PubMed:18817523). Undergoes cysteine S-palmitoylation which promotes interaction with the retromer cargo-selective complex which mediates its retrograde trafficking to the Golgi apparatus (PubMed:18817523).