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Mouse Anti-ERLIN1 Recombinant Antibody (CBXS-2033) (CBMAB-S4800-CQ)

This product is a mouse antibody that recognizes ERLIN1. The antibody CBXS-2033 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all ERLIN1 antibodies

Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
CBXS-2033
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Human
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
ER Lipid Raft Associated 1
Introduction
The protein encoded by this gene is part of a protein complex that mediates degradation of inositol 1,4,5-trisphosphate receptors in the endoplasmic reticulum. The encoded protein also binds cholesterol and regulates the SREBP signaling pathway, which promotes cellular cholesterol homeostasis. Defects in this gene have been associated with spastic paraplegia 62.
Entrez Gene ID
Human10613
Mouse226144
Rat293939
UniProt ID
HumanO75477
MouseQ91X78
RatB1WBY7
Alternative Names
ER Lipid Raft Associated 1; Band_7 23-211 Keo4 (Interim) Similar To C.Elegans Protein C42C1.9; Stomatin-Prohibitin-Flotillin-HflC/K Domain-Containing Protein 1; Endoplasmic Reticulum Lipid Raft-Associated Protein 1; SPFH Domain-Containing Protein 1; SPFH Domain Family, Member 1; C10orf69; SPFH1;
Research Area
Component of the ERLIN1/ERLIN2 complex which mediates the endoplasmic reticulum-associated degradation (ERAD) of inositol 1,4,5-trisphosphate receptors (IP3Rs). Involved in regulation of cellular cholesterol homeostasis by regulation the SREBP signaling pathway. Binds cholesterol and may promote ER retention of the SCAP-SREBF complex (PubMed:24217618).

(Microbial infection) Required early in hepatitis C virus (HCV) infection to initiate RNA replication, and later in the infection to support infectious virus production.
Biological Process
Cholesterol metabolic process Source: UniProtKB-KW
Negative regulation of cholesterol biosynthetic process Source: UniProtKB
Negative regulation of fatty acid biosynthetic process Source: UniProtKB
SREBP signaling pathway Source: UniProtKB
Ubiquitin-dependent ERAD pathway Source: UniProtKB
Cellular Location
Endoplasmic reticulum membrane. Associated with lipid raft-like domains of the endoplasmic reticulum membrane.
Involvement in disease
Spastic paraplegia 62, autosomal recessive (SPG62):
A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.
Topology
Cytoplasmic: 1-7
Helical: 8-28
Lumenal: 29-348

Zhu, Z. Y., Li, Z. Y., Zhang, C., Liu, X. L., Tian, W. T., & Cao, L. (2022). A novel homozygous mutation in ERLIN1 gene causing spastic paraplegia 62 and literature review. European Journal of Medical Genetics, 104608.

Gao, X., Bonzerato, C. G., & Wojcikiewicz, R. J. (2022). Binding of the erlin1/2 complex to the third intralumenal loop of IP3R1 triggers its ubiquitin-proteasomal degradation. Journal of Biological Chemistry, 102026.

Manganelli, V., Matarrese, P., Antonioli, M., Gambardella, L., Vescovo, T., Gretzmeier, C., ... & Garofalo, T. (2021). Raft-like lipid microdomains drive autophagy initiation via AMBRA1-ERLIN1 molecular association within MAMs. Autophagy, 17(9), 2528-2548.

Huang, S. S., Toufiq, M., Saraiva, L. R., Van Panhuys, N., Chaussabel, D., & Garand, M. (2021). Transcriptome and literature mining highlight the differential expression of ERLIN1 in immune cells during sepsis. Biology, 10(8), 755.

Sleptcov, A., Nazarenko, M., Kazantsev, A., Lebedev, I., Barbarash, O., & Puzyrev, V. (2019). Assessment Of Sfmbt1, Prkra, And Erlin1 Copy Number Variations In Patients With Carotid Atherosclerosis Using Droplet Digital Pcr. Atherosclerosis, 287, e165-e166.

Whitten-Bauer, C., Chung, J., Gómez-Moreno, A., Gomollón-Zueco, P., Huber, M. D., Gerace, L., & Garaigorta, U. (2019). The host factor erlin-1 is required for efficient hepatitis C virus infection. Cells, 8(12), 1555.

Tunca, C., Akçimen, F., Coşkun, C., Gündoğdu-Eken, A., Kocoglu, C., Çevik, B., ... & Başak, A. N. (2018). ERLIN1 mutations cause teenage-onset slowly progressive ALS in a large Turkish pedigree. European Journal of Human Genetics, 26(5), 745-748.

Wright, F. A., Bonzerato, C. G., Sliter, D. A., & Wojcikiewicz, R. J. (2018). The erlin2 T65I mutation inhibits erlin1/2 complex–mediated inositol 1, 4, 5-trisphosphate receptor ubiquitination and phosphatidylinositol 3-phosphate binding. Journal of Biological Chemistry, 293(40), 15706-15714.

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For research use only. Not intended for any clinical use.

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