NUP98

(redirected from GLFG-repeat containing nucleoporin)

NUP98

A gene on chromosome 11p15.5 that encodes a nucleoporin (one of the estimated 50 nucleoporins), which derives from cleavage of a 186-kDa precursor and functions as one of several docking site nucleoporins of transport substrates.

Molecular pathology
NUP98 fuses to several genes after chromosome translocations in acute myeloid leukaemia (AML) and T-cell acute lymphocytic leukaemia (T-ALL), and is one of several genes located in the imprinted gene domain of 11p15.5, a tumour-suppressor gene region. Alterations in 11p15.5 region are associated with the Beckwith-Wiedemann syndrome, Wilms tumour, rhabdomyosarcoma and adrenocortical carcinoma, as well as lung, ovarian and breast cancers.