Giant axonal neuropathy: a conditional mutation affecting cytoskeletal organization
摘要：
Giant axonal neuropathy (GAN) results from autosomal recessive mutations (gan-) that affect cytoskeletal organization; specifically, intermediate filaments (IFs) are found collapsed into massive bundles in a variety of different cell types. We studied the gan- fibroblast lines WG321 and WG139 derived from different GAN patients. Although previous studies implied that the gan- IF phenotype was constitutive, we find that it is conditional. That is, when cells were grown under the permissive condition of medium containing over 2% fetal calf serum, most cells had normal IF organization. IF bundles formed when gan- cells were transferred to the nonpermissive condition of low (0.1%) serum. Microtubule organization appeared normal in the presence or absence of serum. The effect of serum starvation was largely blocked or reversed by the addition of BSA to the culture media. We found no evidence that the gan- phenotype depends upon progress through the cell cycle. We discuss the possible role of serum effects in the etiology of GAN and speculate as to the molecular nature of the gan- defect.
展开
DOI：
10.1083/jcb.100.1.245
被引量：
年份：
1985
Semantic Scholar
(全网免费下载)
掌桥科研
Semantic Scholar
万方医学
dx.doi.org
查看更多
Pubmed Central
(全网免费下载)
Europe PMC
(全网免费下载)
NCBI
Europe PMC
NCBI
ResearchGate
ResearchGate
(全网免费下载)
EureKaMag.com
(全网免费下载)
ProQuest
Citeseer
(全网免费下载)
EBSCO
biomedsearch.com
(全网免费下载)
钛学术
jcb.rupress.org
(全网免费下载)
jcb.rupress.org
rupress.org
scienceopen.com
onacademic.com
invenzone.com
core.ac.uk
mendeley.com
deepdyve.com
钛学术
(全网免费下载)
www.socolar.com
Semantic Scholar
(全网免费下载)
Europe PMC
(全网免费下载)
Pubmed Central
(全网免费下载)
ResearchGate
(全网免费下载)
EureKaMag.com
(全网免费下载)
Citeseer
(全网免费下载)
biomedsearch.com
(全网免费下载)
钛学术
(全网免费下载)
jcb.rupress.org
(全网免费下载)
通过文献互助平台发起求助，成功后即可免费获取论文全文。
相似文献
参考文献
引证文献
辅助模式
引用
文献可以批量引用啦~
欢迎点我试用！
