Pericentrin in cellular function and disease

被引:138
作者
Delaval, Benedicte [1 ]
Doxsey, Stephen J. [1 ]
机构
[1] Univ Massachusetts, Sch Med, Program Mol Med, Worcester, MA 01605 USA
基金
美国国家卫生研究院;
关键词
CYSTIC KIDNEY-DISEASE; SPINDLE POLE BODY; MICROTUBULE-ORGANIZING CENTERS; CEPHALIC PRIMORDIAL DWARFISM; MYELOID-LEUKEMIA CORRELATE; GAMMA-TUBULIN COMPLEXES; SECKEL-SYNDROME; CENTROSOME ABERRATIONS; MITOTIC SPINDLE; GENETIC INSTABILITY;
D O I
10.1083/jcb.200908114
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Pericentrin is an integral component of the centrosome that serves as a multifunctional scaffold for anchoring numerous proteins and protein complexes. Through these interactions, pericentrin contributes to a diversity of fundamental cellular processes. Recent studies link pericentrin to a growing list of human disorders. Studies on pericentrin at the cellular, molecular, and, more recently, organismal level, provide a platform for generating models to elucidate the etiology of these disorders. Although the complexity of phenotypes associated with pericentrin-mediated disorders is somewhat daunting, insights into the cellular basis of disease are beginning to come into focus. In this review, we focus on human conditions associated with loss or elevation of pericentrin and propose cellular and molecular models that might explain them.
引用
收藏
页码:181 / 190
页数:10
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