Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=25722189
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\25722189
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Dev+Biol
2015 ; 400
(2
): 248-57
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
IFT46 plays an essential role in cilia development
#MMPMID25722189
Lee MS
; Hwang KS
; Oh HW
; Ji-Ae K
; Kim HT
; Cho HS
; Lee JJ
; Yeong Ko J
; Choi JH
; Jeong YM
; You KH
; Kim J
; Park DS
; Nam KH
; Aizawa S
; Kiyonari H
; Shioi G
; Park JH
; Zhou W
; Kim NS
; Kim CH
Dev Biol
2015[Apr]; 400
(2
): 248-57
PMID25722189
show ga
Cilia are microtubule-based structures that project into the extracellular space.
Ciliary defects are associated with several human diseases, including polycystic
kidney disease, primary ciliary dyskinesia, left-right axis patterning,
hydrocephalus and retinal degeneration. However, the genetic and cellular
biological control of ciliogenesis remains poorly understood. The IFT46 is one of
the highly conserved intraflagellar transport complex B proteins. In zebrafish,
ift46 is expressed in various ciliated tissues such as Kupffer?s vesicle,
pronephric ducts, ears and spinal cord. We show that ift46 is localized to the
basal body. Knockdown of ift46 gene results in multiple phenotypes associated
with various ciliopathies including kidney cysts, pericardial edema and ventral
axis curvature. In ift46 morphants, cilia in kidney and spinal canal are
shortened and abnormal. Similar ciliary defects are observed in otic vesicles,
lateral line hair cells, olfactory pits, but not in Kupffer?s vesicle. To explore
the functions of Ift46 during mouse development, we have generated Ift46
knock-out mice. The Ift46 mutants have developmental defects in brain, neural
tube and heart. In particular Ift46(-/-) homozygotes displays randomization of
the embryo heart looping, which is a hallmark of defective left-right (L/R) axis
patterning. Taken together, our results demonstrated that IFT46 has an essential
role in vertebrate ciliary development.
|Amino Acid Sequence
[MESH]
|Animals
[MESH]
|Basal Bodies/metabolism
[MESH]
|Cilia/*metabolism
[MESH]
|Cytoskeletal Proteins
[MESH]
|Gene Expression
[MESH]
|Humans
[MESH]
|Intracellular Signaling Peptides and Proteins/genetics/*metabolism
[MESH]
free
free
free
