Domain

Tumor necrosis factor receptor 11A, N-terminal (IPR034040)

Short name: TNFRSF11A_N

Overlapping homologous superfamilies

None.

Domain relationships

None.

Description

Tumor necrosis factor receptor superfamily member 11A (TNFRSF11A), also known as RANK, FEO, OFE, ODFR, OSTS, PDB2, CD26, OPTB7, TRANCER, or LOH18CR1, induces the activation of NF-kappa B and MAPK8/JNK through interactions with various TRAF adaptor proteins [PMID: 17633024]. This receptor and its ligand are important regulators of the interaction between T cells and dendritic cells. The receptor is also an essential mediator for osteoclast and lymph node development. Mutations at this locus have been associated with familial expansile osteolysis, autosomal recessive osteopetrosis, and Juvenile Paget's disease (JPD) of bone [PMID: 18328984, PMID: 21059944, PMID: 20564239, PMID: 25063546]. Alternatively spliced transcript variants have been described for this locus [PMID: 23664977]. Mutation analysis may improve diagnosis, prognostication, recurrence risk assessment, and perhaps treatment selection among the monogenic disorders of RANKL/OPG/RANK activation.

This entry represents the N-terminal domain of TNFRSF11A. TNF-receptors are modular proteins. The N-terminal extracellular part contains a cysteine-rich region responsible for ligand-binding. This region is composed of small modules of about 40 residues containing 6 conserved cysteines; the number and type of modules can vary in different members of the family [PMID: 9538693, PMID: 8387891, PMID: 11796220].

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
CDD