The ciliary transition zone: from morphology and molecules to medicine

被引：104
作者：
Czarnecki, Peter G. [1 ,2 ]
Shah, Jagesh V. [1 ,3 ]
机构：
[1] Harvard Univ, Sch Med, Dept Syst Biol, Boston, MA 02215 USA
[2] Beth Israel Deaconess Med Ctr, Div Nephrol, Boston, MA 02215 USA
[3] Brigham & Womens Hosp, Div Renal, Boston, MA 02115 USA
来源：
TRENDS IN CELL BIOLOGY | 2012年 / 22卷 / 04期
基金：
美国国家卫生研究院;
关键词：
MECKEL-GRUBER-SYNDROME; BASAL BODY PROTEOME; HUMAN-DISEASE GENE; JOUBERT-SYNDROME; PRIMARY CILIUM; KIDNEY-DISEASE; CAENORHABDITIS-ELEGANS; SENSORY CILIA; CAUSE NEPHRONOPHTHISIS; RETINAL DEGENERATION;
D O I：
10.1016/j.tcb.2012.02.001
中图分类号：
Q2 [细胞生物学];
学科分类号：
071009 ; 090102 ;
摘要：
Researchers from various disciplines, including cell and developmental biology, genetics and molecular medicine, have revealed an exceptional diversity of cellular functions that are mediated by cilia-dependent mechanisms. Recent studies have directed our attention to proteins that localize to the ciliary transition zone (TZ), a small evolutionarily conserved subcompartment that is situated between the basal body (BB) and the more distal ciliary axoneme. These reports shed light on the roles of TZ proteins in ciliogenesis, ciliary protein homeostasis and specification of ciliary signaling, and pave the way for understanding their contribution to human ciliopathies. In this review, we describe the interplay of multimeric protein complexes at the TZ, integrating morphological, genetic and proteomic data towards an account of TZ function in ciliary physiology.
引用
收藏
页码：201 / 210
页数：10
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