Ubiquitin ligase SYVN1/HRD1 facilitates degradation of the SERPINA1 Z variant/α-1-antitrypsin Z variant via SQSTM1/p62-dependent selective autophagy

SERPINA1/AAT/α-1-antitrypsin (serpin family A member 1) deficiency (SERPINA1/ AAT-D) is an autosomal recessive disorder characterized by the retention of misfolded SERPINA1/AAT in the endoplasmic reticulum (ER) of hepatocytes and a significant reduction of serum SERPINA1/AAT level. The Z variant of...

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Detalles Bibliográficos
Autores principales: Feng, Lijie, Zhang, Jin, Zhu, Na, Ding, Qian, Zhang, Xiaojie, Yu, Jishuang, Qiang, Weimin, Zhang, Zhetao, Ma, Yuyang, Huang, Dake, Shen, Yujun, Fang, Shengyun, Yu, Yifan, Wang, Haiping, Shen, Yuxian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2017
Materias:
Basic Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5388218/
https://www.ncbi.nlm.nih.gov/pubmed/28121484
http://dx.doi.org/10.1080/15548627.2017.1280207

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