KEO4 _ ERLIN1
- Known as:
- KEO4 _ ERLIN1
- Catalog number:
- Y214291
- Product Quantity:
- 200ul
- Category:
- -
- Supplier:
- ABM
- Gene target:
- KEO4 _ ERLIN1
Related genes to: KEO4 _ ERLIN1
- Gene:
- ERLIN1 NIH gene
- Name:
- ER lipid raft associated 1
- Previous symbol:
- C10orf69, SPFH1
- Synonyms:
- KE04, Erlin-1, SPG62
- Chromosome:
- 10q24.31
- Locus Type:
- gene with protein product
- Date approved:
- 2004-02-05
- Date modifiied:
- 2014-11-19
Related products to: KEO4 _ ERLIN1
Antibodies: KEO4 _ ERLIN1 HOST: Goat Clonality: pAbC10orf69,Endoplasmic reticulum lipid raft-associated protein 1,ERLIN1,Erlin-1,Homo sapiens,Human,KE04,KEO4,Protein KE04,SPFH domain-containing protein 1,SPFH1,Stomatin-prohibitin-flotillin-HflC_K domaELISA Kit for ER Lipid Raft Associated Protein 1 (ERLIN1)ELISA Kit for ER Lipid Raft Associated Protein 1 (ERLIN1) Homo sapiens (Human)ELISA Kit for ER Lipid Raft Associated Protein 1 (ERLIN1) Organism: Homo sapiens (Human)ELISA Kit FOR Erlin-1; organism: Mouse; gene name: Erlin1Endoplasmic reticulum lipid raft-associated protein 1,Erlin1,Erlin-1,Keo4,Mouse,Mus musculus,Protein KE04 homolog,SPFH domain-containing protein 1,Spfh1,Stomatin-prohibitin-flotillin-HflC_K domain-conERICH1 Gene glutamate-rich 1ERLIN1Erlin1ERLIN1Erlin1ERLIN1 3&_39;UTR Lenti-reporter-Luc VectorERLIN1 antibodyERLIN1 antibody Related articles to: KEO4 _ ERLIN1
- The SPFH (stomatin, prohibitin, flotillin, and HflK/C) family proteins are proposed scaffolds for organizing functional membrane microdomains (FMMs) on various cellular membranes. Erlin1 and Erlin2, two endoplasmic reticulum (ER)-residing SPFH members, as heteromeric complexes, participate in ER-associated protein degradation (ERAD). However, the mechanisms underlying Erlin-mediated FMM organization and ERAD regulation remain poorly understood. Here, through cryoelectron microscopy (cryo-EM), we find that the human Erlin1/2 complex forms a 26-mer cage assembly, defining a nanometer-sized microdomain on the luminal leaflet. The intramembrane region of each subunit constitutes a specific phosphatidylinositol-binding pocket. ER proteins can be recruited to both the interior and exterior of these cages. By caging cargoes, the Erlin1/2 complex physically secludes them from their substrates or binding partners, conferring another layer of regulation on their functions. Moreover, individual cages can cluster to organize FMMs of different sizes. These dynamic properties underscore a general regulatory role of Erlin1/2 in various ER-related biological processes, including coronaviral replication. - Source: PubMed
Publication date: 2026/03/25
Yan LuXu ZihongYao YuanhangAwang TadsaneeWang XiaotingWang YonglunMa ChengyingLi NingningSong ChenChen Xiao-WeiGao Ning - Dysregulated cholesterol metabolism is a hallmark of hepatocellular carcinoma (HCC) that drives tumor initiation and progression. However, clinical targeting of cholesterol metabolism has yielded limited benefits due to stringent feedback in tumor cells. Identifying a central mediator capable of restoring cholesterol homeostasis within the cell's intrinsically fine-tuned regulatory framework is urgently needed. - Source: PubMed
Publication date: 2026/02/11
Zhang YimingHou YushanWang XinxinXu KaikunJiang PeiWang SiqiKang HuiminZhang HuJin JingzhuoHuang XiaofenLiu ZifengYang SongpengLiu JiaqiZhang LingqiangHe FuchuTian ChunyanSun Aihua - Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease marked by progressive degeneration of upper and lower motor neurons. Most forms of ALS associated with a suspected causal variant are inherited in an autosomal dominant manner. However, there is an important subset of autosomal recessive (AR) variants, often associated with early-onset or atypical clinical features. Advances in genetic sequencing have led to increased recognition of AR ALS. In this review, we focus on four key confirmed AR ALS-associated genes, which appear to be most common-, , , and the D90A variant of -reviewing their pathophysiology and unique clinical manifestations. We also highlight very rare AR mutations implicated in ALS, including , , and , and some associated with overlap syndromes or debated pathogenicity including , , and These genes are involved in an array of processes including axonal transport, endosomal trafficking, oxidative stress response, and autophagy, suggesting distinct mechanisms of motor neuron degeneration. Some forms of AR ALS more frequently present with juvenile onset and slower progression, but other genes are associated with broader phenotypic spectra. This includes overlap with hereditary spastic paraplegia (HSP) and hereditary ataxias. Understanding these AR forms of ALS may enhance diagnostic precision, improve prognostication, and may pave the way for targeted gene therapies. This review underscores the emerging significance of AR inheritance in ALS and calls for deeper investigation into its molecular and clinical dimensions. - Source: PubMed
Publication date: 2026/01/27
Allen Matti DDiab VanessaLezaic NastasijaBinet MayaGentil Benoit JBlanchard OliverGenge AngelaMassie Rami - The endoplasmic reticulum (ER) lipid raft proteins (Erlins) belong to the stomatin-prohibitin-flotillin-HflC/K (SPFH) family and form highly oligomeric platforms that mediate the degradation of activated inositol 1,4,5-trisphosphate receptors by facilitating their interaction with the E3 ligase RNF170. However, the molecular mechanisms underlying this process remain unclear. Here, we successfully reconstituted the Erlin1-Erlin2 complex and its complex with RNF170 by overexpressing these components in HEK293F cells. We also isolated the Erlin2 oligomer by solely expressing Erlin2 in the cells. Using cryo-EM, we determined the structures of the Erlin1-Erlin2 complex, Erlin1-Erlin2-RNF170 complex, and Erlin2 oligomer at resolutions of 3.29 Å, 3.05 Å, and 2.12 Å, respectively. Both the Erlin1-Erlin2 complex and the Erlin2 oligomer exhibit similar cage-like architectures, with the Erlin1-Erlin2 complex containing 13 pairs of Erlin1 and Erlin2 subunits, whereas the Erlin2 oligomer comprises 26 Erlin2. Although RNF170 was clearly identified during protein purification, it was invisible in the final 3D reconstruction, suggesting a high degree of flexibility between RNF170 and the Erlin complex. Multiple water molecules were identified in the Erlin2 oligomer, underscoring their critical roles in facilitating the high degree of oligomerization of the Erlin2 complex. Taken together, our structural investigation elucidates the molecular basis for the assembly of the Erlin complex and provides a framework for further investigation. - Source: PubMed
Publication date: 2026/01/02
Jia XiaoxiaoLiu GuoyunLi HaiwenQian Hongwu - Hereditary spastic paraplegia type 62 (SPG62) is a neurodegenerative disorder, with more than 20 individuals reported to date. This ultra-rare entity is inherited in an autosomal recessive manner and has been associated with ERLIN1 variants. In addition, ERLIN1-related biallelic variants have been linked to early-onset amyotrophic lateral sclerosis (ALS). We present two siblings with slowly progressive spastic paraplegia, with mild intellectual decline, behavioral findings, and hyperacusis. This study expands the clinical spectrum of hereditary spastic paraplegia associated with ERLIN1. - Source: PubMed
Publication date: 2025/07/21
Ozkose Gulsah SebnemTopcu YaseminAy BerilOzdemir OzkanAkgun-Dogan OzlemNg Ozden HatirnazAlanay Yasemin