ER-phagy: mechanisms, regulation, and diseases connected to the lysosomal clearance of the endoplasmic reticulum

F Reggiori, M Molinari - Physiological reviews, 2022 - journals.physiology.org
ER-phagy (reticulophagy) defines the degradation of portions of the endoplasmic reticulum
(ER) within lysosomes or vacuoles. It is part of the self-digestion (ie, autophagic) programs …

The UFMylation system in proteostasis and beyond

Y Gerakis, M Quintero, H Li, C Hetz - Trends in cell biology, 2019 - cell.com
Post-translational modifications are at the apex of cellular communication and eventually
regulate every aspect of life. The identification of new post-translational modifiers is opening …

[HTML][HTML] Ubiquitin fold modifier 1 (UFM1) and its target UFBP1 protect pancreatic beta cells from ER stress-induced apoptosis

K Lemaire, RF Moura, M Granvik, M Igoillo-Esteve… - PloS one, 2011 - journals.plos.org
UFM1 is a member of the ubiquitin like protein family. While the enzymatic cascade of UFM1
conjugation has been elucidated in recent years, the biological function remains largely …

[HTML][HTML] RCAD/Ufl1, a Ufm1 E3 ligase, is essential for hematopoietic stem cell function and murine hematopoiesis

M Zhang, X Zhu, Y Zhang, Y Cai, J Chen… - Cell Death & …, 2015 - nature.com
The Ufm1 conjugation system is a novel ubiquitin-like modification system, consisting of
Ufm1, Uba5 (E1), Ufc1 (E2) and poorly characterized E3 ligase (s). RCAD/Ufl1 (also known …

[HTML][HTML] Ufbp1 promotes plasma cell development and ER expansion by modulating distinct branches of UPR

H Zhu, B Bhatt, S Sivaprakasam, Y Cai, S Liu… - Nature …, 2019 - nature.com
The IRE1α/XBP1 branch of unfolded protein response (UPR) pathway has a critical function
in endoplasmic reticulum (ER) expansion in plasma cells via unknown mechanisms; …

[HTML][HTML] Transcriptional regulation of the Ufm1 conjugation system in response to disturbance of the endoplasmic reticulum homeostasis and inhibition of vesicle …

Y Zhang, M Zhang, J Wu, G Lei, H Li - PloS one, 2012 - journals.plos.org
Homeostasis of the endoplasmic reticulum (ER) is essential for normal cellular functions.
Disturbance of this homeostasis causes ER stress and activates the Unfolded Protein …

[PDF][PDF] Biallelic variants in UBA5 reveal that disruption of the UFM1 cascade can result in early-onset encephalopathy

E Colin, J Daniel, A Ziegler, J Wakim, A Scrivo… - The American Journal of …, 2016 - cell.com
Via whole-exome sequencing, we identified rare autosomal-recessive variants in UBA5 in
five children from four unrelated families affected with a similar pattern of severe intellectual …

Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1

M Cvetanovic, M Ingram, H Orr, P Opal - Neuroscience, 2015 - Elsevier
Abstract Spinocerebellar ataxia type 1 (SCA1) is an incurable, dominantly inherited
neurodegenerative disease of the cerebellum caused by a polyglutamine-repeat expansion …

The role of astrocytes in the development of the cerebellum

APB Araujo, R Carpi-Santos, FCA Gomes - The Cerebellum, 2019 - Springer
Astrocytes, initially described as merely support cells, are now known as a heterogeneous
population of cells actively involved in a variety of biological functions such as: neuronal …

[HTML][HTML] UBA5 mutations cause a new form of autosomal recessive cerebellar ataxia

R Duan, Y Shi, L Yu, G Zhang, J Li, Y Lin, J Guo… - PLoS …, 2016 - journals.plos.org
Autosomal recessive cerebellar ataxia (ARCA) comprises a large and heterogeneous group
of neurodegenerative disorders. For many affected patients, the genetic cause remains …