Hypomorphic variants of SEL1L-HRD1 ER-associated degradation are associated with neurodevelopmental disorders
Recent studies using cell type–specific knockout mouse models have improved our
understanding of the pathophysiological relevance of suppressor of lin-12-like–HMG-CoA …
understanding of the pathophysiological relevance of suppressor of lin-12-like–HMG-CoA …
Proteomic screens of SEL1L-HRD1 ER-associated degradation substrates reveal its role in glycosylphosphatidylinositol-anchored protein biogenesis
Endoplasmic reticulum-associated degradation (ERAD) plays indispensable roles in many
physiological processes; however, the nature of endogenous substrates remains largely …
physiological processes; however, the nature of endogenous substrates remains largely …
Diversity and structural‐functional insights of alpha‐solenoid proteins
Alpha‐solenoids are a significant and diverse subset of structured tandem repeat proteins
(STRPs) that are important in various domains of life. This review examines their structural …
(STRPs) that are important in various domains of life. This review examines their structural …
SEL1L-HRD1 interaction is required to form a functional HRD1 ERAD complex
Abstract The SEL1L-HRD1 protein complex represents the most conserved branch of
endoplasmic reticulum (ER)-associated degradation (ERAD). Despite recent advances in …
endoplasmic reticulum (ER)-associated degradation (ERAD). Despite recent advances in …
Regulation of leptin signaling and diet-induced obesity by SEL1L-HRD1 ER-associated degradation in POMC expressing neurons
Endoplasmic reticulum (ER) homeostasis in the hypothalamus has been implicated in the
pathogenesis of diet-induced obesity (DIO) and type 2 diabetes; however, the underlying …
pathogenesis of diet-induced obesity (DIO) and type 2 diabetes; however, the underlying …
Regulation of hepatic inclusions and fibrinogen biogenesis by SEL1L-HRD1 ERAD
Z Song, P Thepsuwan, WS Hur, M Torres… - Nature …, 2024 - nature.com
Impaired secretion of an essential blood coagulation factor fibrinogen leads to hepatic
fibrinogen storage disease (HFSD), characterized by the presence of fibrinogen-positive …
fibrinogen storage disease (HFSD), characterized by the presence of fibrinogen-positive …
A pipeline for senolytics
S Khosla - The Journal of Clinical Investigation, 2024 - Am Soc Clin Investig
There is intense interest in identifying compounds that selectively kill senescent cells,
termed senolytics, for ameliorating age-related comorbidities. However, screening for …
termed senolytics, for ameliorating age-related comorbidities. However, screening for …
Genome-wide screens identify SEL1L as an intracellular rheostat controlling collagen turnover
MJ Podolsky, B Kheyfets, M Pandey, AH Beigh… - Nature …, 2024 - nature.com
Accumulating evidence has implicated impaired extracellular matrix (ECM) clearance as a
key factor in fibrotic disease. Despite decades of research elucidating the effectors of ECM …
key factor in fibrotic disease. Despite decades of research elucidating the effectors of ECM …
[HTML][HTML] Purkinje cell–specific deficiency in SEL1L-HRD1 endoplasmic reticulum–associated degradation causes progressive cerebellar ataxia in mice
M Torres, B Pederson, H Wang, LL Lin, HH Wang… - JCI …, 2024 - pmc.ncbi.nlm.nih.gov
Recent studies have identified multiple genetic variants of SEL1L-HRD1 endoplasmic
reticulum–associated degradation (ERAD) in humans with neurodevelopmental disorders …
reticulum–associated degradation (ERAD) in humans with neurodevelopmental disorders …
Hypomorphic human SEL1L and HRD1 variants uncouple multilayered ER-associated degradation machinery
K Umphred-Wilson, S Adoro - The Journal of Clinical …, 2024 - Am Soc Clin Investig
The suppressor of lin-12-like–HMG-CoA reductase degradation 1 (SEL1L-HRD1) complex
of the endoplasmic reticulum–associated degradation (ERAD) machinery is a key cellular …
of the endoplasmic reticulum–associated degradation (ERAD) machinery is a key cellular …