Target intelligence / Profile preview

Ran GTPase-activating protein 1 (RANGAP1)

Target
RANGAP1
Molecular classification
Enzyme, GTPase-activating protein, Other (Nuclear trafficking protein)
01

Overview

Ran GTPase-activating protein 1 (RANGAP1) is an enzyme that regulates the activity of the small GTPase Ran by accelerating its intrinsic GTP hydrolysis, thereby converting Ran-GTP to Ran-GDP. This activity is critical in controlling the directionality and dynamics of nucleocytoplasmic transport of proteins and RNA through the nuclear pore complex. RANGAP1 acts as the functional antagonist of RCC1, which serves as the guanine nucleotide exchange factor for Ran. Through its interactions and localization, particularly at the nuclear envelope (after post-translational modification by SUMO), RANGAP1 ensures the proper shuttling of cargo molecules between the nucleus and cytoplasm. Defects in RANGAP1 function can lead to impairment of nucleocytoplasmic transport, impacting processes such as cell cycle progression and gene expression, and have been associated with some human diseases.

Other names
RANGAP1RanGAP1Fug1RANGAPSDsegregation distorter homologKIAA1835
02

Biological functions

Regulation of nuclear transportGTPase activator activity (promotes GTP hydrolysis by Ran)Cell cycle regulationProtein localization to the nuclear envelopeMediator of nucleocytoplasmic transport
03

Disease associations

Parkinson disease (implicated in early-onset forms through disease association)Nephronophthisis-like nephropathyOther (general nuclear trafficking defects; functional defects described in model organisms)

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