PRPF3

The removal of introns from nuclear pre-mRNAs occurs on complexes called spliceosomes, which are made up of 4 small nuclear ribonucleoprotein (snRNP) particles and an undefined number of transiently associated splicing factors. This gene product is one of several proteins that associate with U4 and U6 snRNPs. Mutations in this gene are associated with retinitis pigmentosa-18. [provided by RefSeq]

Full Name

PRP3 pre-mRNA processing factor 3 homolog (S. cerevisiae)

Function

Plays role in pre-mRNA splicing as component of the U4/U6-U5 tri-snRNP complex that is involved in spliceosome assembly, and as component of the precatalytic spliceosome (spliceosome B complex).

Biological Process

mRNA processingManual Assertion Based On ExperimentTAS:ProtInc
mRNA splicing, via spliceosomeManual Assertion Based On ExperimentIDA:UniProtKB
RNA splicingManual Assertion Based On ExperimentTAS:ProtInc
RNA splicing, via transesterification reactionsManual Assertion Based On ExperimentTAS:UniProtKB
Spliceosomal tri-snRNP complex assemblyManual Assertion Based On ExperimentIDA:UniProtKB

Cellular Location

Nucleus
Nucleus speckle

Involvement in disease

Retinitis pigmentosa 18 (RP18):
A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

PTM

Ubiquitinated. Undergoes 'Lys-63'-linked polyubiquitination by PRPF19 and deubiquitination by USP4. 'Lys-63'-linked ubiquitination increases the affinity for PRPF8 and may regulate the assembly of the U4/U6-U5 tri-snRNP complex.