survival of motor neurons;
spinal muscular atrophy;
small nuclear ribonucleoprotein;
unr-interacting protein;
D O I:
10.1016/j.febslet.2005.03.034
中图分类号:
Q5 [生物化学];
Q7 [分子生物学];
学科分类号:
071010 ;
081704 ;
摘要:
A macromolecular complex containing survival of motor neurons (SMN), the spinal muscular atrophy protein, and Gemin2-7 interacts with Sm proteins and snRNAs to carry out the assembly of these components into spliceosomal small nuclear ribonucleoproteins (snRNPs). Here we report the characterization of unr-interacting protein (unrip), a GH-WD protein of unknown function, as a component of the SMN complex that interacts directly with Gemin6 and Gemin7. Unrip also binds a subset of Sm proteins, and unrip-containing SMN complexes are necessary and sufficient to mediate the assembly of spliceosomal snRNPs. These results demonstrate that unrip functions in the pathway of snRNP biogenesis and is a marker of cellular SMN complexes active in snRNP assembly. (c) 2005 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Gubitz, AK
;
Mourelatos, Z
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Mourelatos, Z
;
Abel, L
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Abel, L
;
Rappsilber, J
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Rappsilber, J
;
Mann, M
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Mann, M
;
Dreyfuss, G
论文数: 0引用数: 0
h-index: 0
机构:
Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USAUniv Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Gubitz, AK
;
Mourelatos, Z
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Mourelatos, Z
;
Abel, L
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Abel, L
;
Rappsilber, J
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Rappsilber, J
;
Mann, M
论文数: 0引用数: 0
h-index: 0
机构:Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA
Mann, M
;
Dreyfuss, G
论文数: 0引用数: 0
h-index: 0
机构:
Univ Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USAUniv Penn, Howard Hughes Med Inst, Sch Med, Dept Biochem & Biophys, Philadelphia, PA 19104 USA