Target intelligence / Profile preview

Phosphorylated adaptor for RNA export (PHAX)

Target
PHAX
Molecular classification
RNA-binding protein, Nuclear transport factor, Other
01

Overview

Phosphorylated adaptor for RNA export (PHAX) is an RNA-binding nuclear transport protein essential for the export of small nuclear RNAs (such as U snRNA) from the nucleus to the cytoplasm in metazoans. PHAX acts as a bridge between the cap binding complex (CBC)-bound snRNA and the export receptor XPO1 (also known as CRM1), and its phosphorylation state controls assembly and disassembly of the export complex: phosphorylation in the nucleus is required for export complex assembly and export, while dephosphorylation in the cytoplasm leads to complex disassembly and recycling of PHAX. PHAX is also involved in the intranuclear trafficking and biogenesis of small nucleolar RNA (snoRNA), binding both precursor and mature forms in a cap-dependent manner. Mutations in PHAX are associated with developmental brain disorders, but it is not considered a direct therapeutic drug target.

Other names
PHAXRNUXARNA U, small nuclear RNA export adaptor (phosphorylation regulated)RNA U small nuclear RNA export adapter proteinPhosphorylated adapter RNA export proteinFLJ13193
02

Biological functions

Nuclear export of small nuclear RNA (snRNA)RNA stabilizationBiogenesis and transport of small nucleolar RNA (snoRNA)Interaction with cap binding complex and export receptor proteins
03

Disease associations

Intellectual developmental disorder, autosomal dominant 10Leukodystrophy, demyelinating, adult-onset, autosomal dominant, typicalOther

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