Neuronal SEL1L-HRD1 ERAD regulates one-carbon metabolism and is essential for motor function and survival

ABSTRACT: SEL1L-HRD1 endoplasmic reticulum-associated degradation (ERAD) is essential for protein folding quality control and maintaining homeostasis in the ER. Defects in SEL1L-HRD1 ERAD complex has been linked to early onset of severe neurological syndromes. However, its physiological importance and underlying mechanisms in neurons remain poorly understood. This study established a mouse model with neuron-specific deletion of Sel1L and revealed that neuronal SEL1L-HRD1 ERAD is essential for maintaining one-carbon metabolism, motor function, and overall viability.

ORGANISM(S): Mus musculus

PROVIDER: GSE299894 | GEO | 2026/02/10

REPOSITORIES: GEO