التفاصيل البيبلوغرافية
| العنوان: |
ATG14 and RB1CC1 play essential roles in maintaining muscle homeostasis |
| المؤلفون: |
Dongfang Li (298810), Peter Vogel (238749), Xiujie Li-Harms (4716744), Bo Wang (86769), Mondira Kundu (696499) |
| سنة النشر: |
2021 |
| المجموعة: |
Smithsonian Institution: Digital Repository |
| مصطلحات موضوعية: |
Biochemistry, Cell Biology, Genetics, Molecular Biology, Neuroscience, Physiology, Developmental Biology, Science Policy, Astronomical and Space Sciences not elsewhere classified, ATG14, autophagy, autophagic vacuolar myopathy, cardiomyopathy, inclusion body myopathy, RB1CC1, RNA-binding protein, ULK1, ULK2 |
| الوصف: |
Defects in macroautophagy/autophagy are implicated in the pathogenesis of neuromuscular and heart diseases. To precisely define the roles of autophagy-related genes in skeletal and cardiac muscles, we generated muscle-specific rb1cc1- and atg14- conditional knockout (cKO) mice by using Ckm/Ckmm2-Cre and compared their phenotypes to those of ulk1 ulk2 -conditional double-knockout (cDKO) mice. atg14 -cKO mice developed hypertrophic cardiomyopathy, which was associated with abnormal accumulation of autophagic cargoes in the heart and early mortality. Skeletal muscles of both atg14 -cKO and rb1cc1 -cKO mice showed features of autophagic vacuolar myopathy with ubiquitin + SQSTM1 + deposits, but only those of rb1cc1 -cKO mice showed TARDBP/TDP-43 + pathology and other features of the inclusion body myopathy–like disease we previously described in ulk1 ulk2 -cDKO mice. Herein, we highlight tissue-specific differences between skeletal and cardiac muscles in their reliance on core autophagy proteins and unique roles for ULK1-ULK2 and RB1CC1 among these proteins in the development of TARDBP + pathology. ABBREVIATIONS :AVM: autophagic vacuolar myopathy; cDKO: conditional double knockout; cKO: conditional knockout; H&E: hematoxylin and eosin; IBM: inclusion body myopathy; mtDNA: mitochondrial DNA; PFA: paraformaldehyde; RNP: ribonucleoprotein; TBST: Tris-buffered saline with 0.2% Triton X-100 |
| نوع الوثيقة: |
dataset |
| اللغة: |
unknown |
| العلاقة: |
https://figshare.com/articles/dataset/ATG14_and_RB1CC1_play_essential_roles_in_maintaining_muscle_homeostasis/14363463Test |
| DOI: |
10.6084/m9.figshare.14363463.v3 |
| الإتاحة: |
https://doi.org/10.6084/m9.figshare.14363463.v3Test |
| حقوق: |
CC BY 4.0 |
| رقم الانضمام: |
edsbas.8B64D055 |
| قاعدة البيانات: |
BASE |
