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排序方式: 共有413条查询结果,搜索用时 15 毫秒
411.
412.
Feline W Lindhout Sybren Portegies Robbelien Kooistra Lotte J Herstel Riccardo Stucchi Jessica J A Hummel Nicky Scheefhals Eugene A Katrukha Maarten Altelaar Harold D MacGillavry Corette J Wierenga Casper C Hoogenraad 《The EMBO journal》2021,40(10)
Axon formation critically relies on local microtubule remodeling and marks the first step in establishing neuronal polarity. However, the function of the microtubule‐organizing centrosomes during the onset of axon formation is still under debate. Here, we demonstrate that centrosomes play an essential role in controlling axon formation in human‐induced pluripotent stem cell (iPSC)‐derived neurons. Depleting centrioles, the core components of centrosomes, in unpolarized human neuronal stem cells results in various axon developmental defects at later stages, including immature action potential firing, mislocalization of axonal microtubule‐associated Trim46 proteins, suppressed expression of growth cone proteins, and affected growth cone morphologies. Live‐cell imaging of microtubules reveals that centriole loss impairs axonal microtubule reorganization toward the unique parallel plus‐end out microtubule bundles during early development. We propose that centrosomes mediate microtubule remodeling during early axon development in human iPSC‐derived neurons, thereby laying the foundation for further axon development and function. 相似文献
413.
Pyruvate dehydrogenase (PDH) E1α is a key component in the PDH complex which catalyzes the oxidative decarboxylation of pyruvate
to acetyl-CoA. Defects in the gene coding for PDH E1α (PDHA1) are associated with a variety of clinical symptoms, often of
a severe character. In the present study, the segregation of three polymorphic CA repeats located in PDHA1 was followed in
the 40 CEPH reference pedigrees. Using these data, multipoint linkage analysis was carried out, refining the genetic location
of PDHA1. The 16-point map presented locates PDHA1 in an approximately 3-cM interval between DXS999 and DXS365 with odds of
more than 1000 : 1. From known physical localizations of the flanking marker loci, PDHA1 could be regionally assigned to Xp22.1-p22.2.
The information provided should be of value in clinical settings.
Received: 10 May 1996 相似文献