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Article

Functional integrity of the SEL1L-HRD1 complex is critical for endoplasmic reticulum-associated degradation and organismal viability.

Proceedings of the National Academy of Sciences of the United States of America - 10 Feb 2026

Zhang Xiawei, Lin Liangguang Leo, Pan Linxiu, Wei Xiaoqiong, Wang Huilun Helen, Li Zexin Jason, Qi Ling

Abstract excerpt

The SEL1L-HRD1 complex is the most conserved branch of endoplasmic reticulum-associated degradation (ERAD), yet whether SEL1L is strictly required for HRD1 function in mammals has remained unclear. Here, we show, using complementary in vivo and in vitro approaches, that direct SEL1L-HRD1 binding is essential for ERAD activity and neonatal survival. Three knock-in mouse models targeting this interface reveal a...

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Identifiers

PMID: 41642983
DOI: 10.1073/pnas.2517927123
PMCID: PMC12891039

Genes

SEL1L

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Functional integrity of the SEL1L-HRD1 complex is critical for endoplasmic reticulum-associated degradation and organismal viability.PMID 41642983Read ↗

Functional integrity of the SEL1L-HRD1 complex is critical for ERAD and organismal viabilitySemantic proximity Read ↗

SEL1L-HRD1 interaction is prerequisite for the formation of a functional HRD1 ERAD complexSemantic proximity Read ↗

Structural basis and pathological implications of the dimeric OS9-SEL1L-HRD1 ERAD Core Complex.Semantic proximity Read ↗

Transcriptomic analysis of SEL1L and HRD1 knockout cell lines reveals multifaceted roles of SEL1L beyond the ER quality controlSemantic proximity Read ↗

Structural basis and pathological implications of the dimeric OS9-SEL1L-HRD1 ERAD Core ComplexSemantic proximity Read ↗

Functional rescue of a disease-linked ERAD pathway mutation via alternative splicing.Semantic proximity Read ↗

A positive genetic selection for transmembrane domain mutations in HRD1 underscores the importance of Hrd1 complex integrity during ERAD.Semantic proximity Read ↗

Endoplasmic reticulum–associated degradation is required for nephrin maturation and kidney glomerular filtration functionSemantic proximity Read ↗

Deficiency of Suppressor Enhancer Lin12 1 Like (SEL1L) in Mice Leads to Systemic Endoplasmic Reticulum Stress and Embryonic LethalitySemantic proximity Read ↗

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