WB: Use at a concentration of 1 - 3 µg/ml. Detects a band of approximately 60 kDa (predicted molecular weight: 55 kDa).
An additional band of unknown identity was also consistently observed at 35kDa. This band was successfully blocked by incubation with the immunising peptide. Also a band of 28kDa was consistently observed, however this band was not blocked by the immunizing peptide and it is therefore a non-specific signal. We call for caution when used for other assays than Western blot.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
機能Involved in pre-mRNA splicing. Required for U4/U6.U5 tri-snRNP formation.
関連疾患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.
配列類似性Belongs to the PRP31 family. Contains 1 Nop domain.
ドメインInteracts with the snRNP via the Nop domain. The coiled coil domain is formed by two non-contiguous helices.
細胞内局在Nucleus speckle. Nucleus > Cajal body. Predominantly found in speckles and in Cajal bodies.