The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500 - 1/1000. Detects a band of approximately 55 kDa (predicted molecular weight: 55 kDa).
Involved in pre-mRNA splicing. Required for U4/U6.U5 tri-snRNP formation.
Involvement in disease
Defects in PRPF31 are the cause of retinitis pigmentosa type 11 (RP11) [MIM:600138]. RP leads to degeneration of retinal photoreceptor cells. 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. RP11 inheritance is autosomal dominant.
Belongs to the PRP31 family. Contains 1 Nop domain.
Interacts with the snRNP via the Nop domain. The coiled coil domain is formed by two non-contiguous helices.
Nucleus speckle. Nucleus > Cajal body. Predominantly found in speckles and in Cajal bodies.