Ribonucleoprotein assembly: Clues from spinal muscular atrophy

被引：26

作者：

Mattaj, IW ^{[1
]}

机构：

[1] European Mol Biol Lab, D-69117 Heidelberg, Germany

来源：

CURRENT BIOLOGY | 1998年 / 8卷 / 03期

关键词：

D O I：

10.1016/S0960-9822(98)70055-7

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：

Mutation or deletion of one of the two genes encoding a protein known as SMN has recently been shown to cause spinal muscular atrophy. The SMN protein has been found to be part of a multi-component complex that appears to function in the assembly of cellular ribonucleoprotein particles. (C) Current Biology Ltd.

引用

页码：R93 / R95

页数：3

共 16 条

[1] When is a deletion not a deletion? When it is converted
Burghes, AHM
[J]. AMERICAN JOURNAL OF HUMAN GENETICS, 1997, 61 (01) : 9 - 15
[2] Genomic variation and gene conversion in spinal muscular atrophy: Implications for disease process and clinical phenotype
Campbell, L
Potter, A
Ignatius, J
Dubowitz, V
Davies, K
[J]. AMERICAN JOURNAL OF HUMAN GENETICS, 1997, 61 (01) : 40 - 50
[3] The survival motor neuron protein in spinal muscular atrophy
Coovert, DD
Le, TT
McAndrew, PE
Strasswimmer, J
Crawford, TO
Mendell, JR
Coulson, SE
Androphy, EJ
Prior, TW
Burghes, AHM
[J]. HUMAN MOLECULAR GENETICS, 1997, 6 (08) : 1205 - 1214
[4] The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis
Fischer, U
Liu, Q
Dreyfuss, G
[J]. CELL, 1997, 90 (06) : 1023 - 1029
[5] Lamond Angus I., 1993, Trends in Cell Biology, V3, P198, DOI 10.1016/0962-8924(93)90214-L
[6] Correlation between severity and SMN protein level in spinal muscular atrophy
Lefebvre, S
Burlet, P
Liu, Q
Bertrandy, S
Clermont, O
Munnich, A
Dreyfuss, G
Melki, J
[J]. NATURE GENETICS, 1997, 16 (03) : 265 - 269
[7] IDENTIFICATION AND CHARACTERIZATION OF A SPINAL MUSCULAR ATROPHY-DETERMINING GENE
LEFEBVRE, S
BURGLEN, L
REBOULLET, S
CLERMONT, O
BURLET, P
VIOLLET, L
BENICHOU, B
CRUAUD, C
MILLASSEAU, P
ZEVIANI, M
LEPASLIER, D
FREZAL, J
COHEN, D
WEISSENBACH, J
MUNNICH, A
MELKI, J
[J]. CELL, 1995, 80 (01) : 155 - 165
[8] The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins
Liu, Q
Fischer, U
Wang, F
Dreyfuss, G
[J]. CELL, 1997, 90 (06) : 1013 - 1021
[9] A novel nuclear structure containing the survival of motor neurons protein
Liu, Q
Dreyfuss, G
[J]. EMBO JOURNAL, 1996, 15 (14) : 3555 - 3565
[10] STRUCTURE OF SPLICEOSOMAL SNRNPS AND THEIR ROLE IN PRE-MESSENGER-RNA SPLICING
LUHRMANN, R
KASTNER, B
BACH, M
[J]. BIOCHIMICA ET BIOPHYSICA ACTA, 1990, 1087 (03) : 265 - 292

← 1 2 →