التفاصيل البيبلوغرافية
| العنوان: |
www.mdpi.com/journal/ijms Loss of Vps54 Function Leads to Vesicle Traffic Impairment, Protein Mis-Sorting and Embryonic Lethality |
| المؤلفون: |
Páll Karlsson, Aida Droce, Jakob M. Moser, Simon Cuhlmann, Carolina Ortiz Padilla, Peter Heimann, Jörg W. Bartsch, Annette Füchtbauer, Ernst-martin Füchtbauer, Thomas Schmitt-john |
| المساهمون: |
The Pennsylvania State University CiteSeerX Archives |
| المصدر: |
http://www.mdpi.com/1422-0067/14/6/10908/pdfTest/. |
| سنة النشر: |
2013 |
| المجموعة: |
CiteSeerX |
| الوصف: |
The identification of the mutation causing the phenotype of the amyotrophic lateral sclerosis (ALS) model mouse, wobbler, has linked motor neuron degeneration with retrograde vesicle traffic. The wobbler mutation affects protein stability of Vps54, a ubiquitously expressed vesicle-tethering factor and leads to partial loss of Vps54 function. Moreover, the Vps54 null mutation causes embryonic lethality, which is associated with extensive membrane blebbing in the neural tube and is most likely a consequence of impaired vesicle transport. Investigation of cells derived from wobbler and Vps54 null mutant embryos demonstrates impaired retrograde transport of the Cholera-toxin B subunit to the trans-Golgi network and mis-sorting of mannose-6-phosphate receptors and cargo proteins dependent on retrograde vesicle transport. Endocytosis assays demonstrate no difference between wobbler and wild type cells, indicating that the retrograde vesicleInt. J. Mol. Sci. 2013, 14 10909 traffic to the trans-Golgi network, but not endocytosis, is affected in Vps54 mutant cells. |
| نوع الوثيقة: |
text |
| اللغة: |
English |
| العلاقة: |
http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.348.4219Test; http://www.mdpi.com/1422-0067/14/6/10908/pdfTest/ |
| الإتاحة: |
http://www.mdpi.com/1422-0067/14/6/10908/pdfTest/ |
| حقوق: |
Metadata may be used without restrictions as long as the oai identifier remains attached to it. |
| رقم الانضمام: |
edsbas.D5F683A7 |
| قاعدة البيانات: |
BASE |
