RANBP2 : RAN binding protein 2
Description
The RANBP2 (RAN binding protein 2) is a protein-coding gene located on chromosome 2.
The RANBP2 gene provides instructions for making a protein that typically associates with a protein complex known as the nuclear pore. The nuclear pore is embedded within the membrane that surrounds the cell's nucleus (called the nuclear envelope), forming a channel that allows transport of molecules in and out of the nucleus. The RANBP2 protein is attached to the nuclear pore outside of the nucleus, where it helps regulate the transport of proteins and other molecules through the nuclear pore and also helps modify proteins coming into or out of the nucleus. When found elsewhere in the cell, the RANBP2 protein plays multiple roles during cell division, including breaking down and forming the nuclear envelope and dividing chromosomes. The RANBP2 protein is thought to associate with cell structures called microtubules, which form scaffolding within the cell to help cells maintain their shape. In conjunction with microtubules, the RANBP2 protein helps transport materials within cells. In nerve cells in the brain, the RANBP2 protein is likely involved in the regulation of energy and the maintenance of the protective barrier that allows only certain substances to pass between blood vessels and the brain (the blood-brain barrier).
E3 SUMO-protein ligase that facilitates SUMO1 and SUMO2 conjugation by UBE2I. It is involved in transport factor (Ran-GTP, karyopherin)-mediated protein import via the F-G repeat-containing domain, which acts as a docking site for substrates. It binds single-stranded RNA (in vitro) and may bind DNA. It is a component of the nuclear export pathway and a specific docking site for the nuclear export factor exportin-1. It inhibits EIF4E-dependent mRNA export. It sumoylates PML at 'Lys-490', which is essential for the proper assembly of PML-NB. It recruits BICD2 to the nuclear envelope and cytoplasmic stacks of nuclear pore complex known as annulate lamellae during G2 phase of cell cycle. It is a probable inactive PPIase with no peptidyl-prolyl cis-trans isomerase activity.
RANBP2 is also known as ADANE, ANE1, IIAE3, NUP358, TRP1, TRP2.
Associated Diseases
- Encephalopathy, acute, infection-induced, susceptibility to, 3
- Familial acute necrotizing encephalopathy
- Acute necrotizing encephalopathy type 1
