Mouse Anti-LSS Recombinant Antibody (2E4) (CBMAB-L2240-YJ)

Name: Mouse Anti-LSS Recombinant Antibody (2E4)
SKU: CBMAB-L2240-YJ
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

2E4

Application

ELISA, IF, IHC

Immunogen

Partial recombinant protein corresponding to aa 633-732 from human LSS (NM_001001438, NP_001001438.1) with GST tag. MW of the GST tag alone is 26kD.

Specificity

Human

Antibody Isotype

IgG1

Clonality

Monoclonal

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!]

Buffer

PBS, pH 7.4

Storage

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

Epitope

aa 633-732

More Infomation

Target

Full Name

lanosterol synthase (2,3-oxidosqualene-lanosterol cyclase)

Introduction

LSS catalyzes the conversion of (S)-2,3 oxidosqualene to lanosterol. LSS is a member of the terpene cyclase/mutase family and catalyzes the first step in the biosynthesis of cholesterol, steroid hormones, and vitamin D. Alternative splicing results in multiple transcript variants encoding different isoforms.

Entrez Gene ID

4047

UniProt ID

P48449

Alternative Names

OSC; CTRCT44; lanosterol synthase; 2,3-epoxysqualene-lanosterol cyclase; hOSC; lanosterol synthase (2,3-oxidosqualene-lanosterol cyclase); oxidosqualene--lanosterol cyclase; EC 5.4.99.7

Function

Key enzyme in the cholesterol biosynthesis pathway. Catalyzes the cyclization of (S)-2,3 oxidosqualene to lanosterol, a reaction that forms the sterol nucleus (PubMed:14766201, PubMed:7639730, PubMed:26200341).
Through the production of lanosterol may regulate lens protein aggregation and increase transparency (PubMed:26200341).

Biological Process

Cholesterol biosynthetic processManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of protein stabilityManual Assertion Based On ExperimentIMP:UniProtKB
Steroid biosynthetic processManual Assertion Based On ExperimentIMP:UniProtKB
Triterpenoid biosynthetic processIEA:InterPro

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Cataract 44 (CTRCT44):
An opacification of the crystalline lens of the eye that frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function.
Hypotrichosis 14 (HYPT14):
A form of hypotrichosis, a condition characterized by the presence of less than the normal amount of hair and abnormal hair follicles and shafts, which are thin and atrophic. The extent of scalp and body hair involvement can be very variable, within as well as between families. HYPT14 is an autosomal recessive form characterized by sparse to absent lanugo-like scalp hair, sparse and brittle eyebrows, and sparse eyelashes and body hair.
Alopecia-intellectual disability syndrome 4 (APMR4):
An autosomal recessive disorder characterized by alopecia universalis, scaly skin, mild to severe intellectual disability, delayed or absent speech, and motor delay.

Sun, X., Zhang, J., Liu, H., Li, M., Liu, L., Yang, Z., ... & Zhang, S. (2023). Lanosterol synthase loss of function decreases the malignant phenotypes of HepG2 cells by deactivating the Src/MAPK signaling pathway. Oncology Letters, 26(1), 1-9.

Ma, P., Huang, J., Chen, B., Huang, M., Xiong, L., Chen, J., ... & Liu, Y. (2023). Lanosterol Synthase Prevents EMT During Lens Epithelial Fibrosis Via Regulating SREBP1. Investigative Ophthalmology & Visual Science, 64(15), 12-12.

Yang, F., Jiang, X., Zhu, Y., Lee, M., Xu, Z., Zhang, J., ... & Lin, Z. (2022). Biallelic variants in lanosterol synthase (LSS) cause palmoplantar keratoderma-congenital alopecia syndrome type 2. Journal of Investigative Dermatology, 142(10), 2687-2694.

Hubler, Z., Friedrich, R. M., Sax, J. L., Allimuthu, D., Gao, F., Rivera-León, A. M., ... & Adams, D. J. (2021). Modulation of lanosterol synthase drives 24, 25-epoxysterol synthesis and oligodendrocyte formation. Cell chemical biology, 28(6), 866-875.

Wada, Y., Kikuchi, A., Kaga, A., Shimizu, N., Ito, J., Onuma, R., ... & Kure, S. (2020). Metabolic and pathologic profiles of human LSS deficiency recapitulated in mice. PLoS Genetics, 16(2), e1008628.

Iatrino, R., Lanzani, C., Bignami, E., Casamassima, N., Citterio, L., Meroni, R., ... & Simonini, M. (2019). Lanosterol synthase genetic variants, endogenous ouabain, and both acute and chronic kidney injury. American Journal of Kidney Diseases, 73(4), 504-512.

Hua, H., Yang, T., Huang, L., Chen, R., Li, M., Zou, Z., ... & Liu, Y. (2019). Protective effects of lanosterol synthase up-regulation in UV-B-induced oxidative stress. Frontiers in Pharmacology, 10, 947.

Besnard, T., Sloboda, N., Goldenberg, A., Küry, S., Cogné, B., Breheret, F., ... & Isidor, B. (2019). Biallelic pathogenic variants in the lanosterol synthase gene LSS involved in the cholesterol biosynthesis cause alopecia with intellectual disability, a rare recessive neuroectodermal syndrome. Genetics in Medicine, 21(9), 2025-2035.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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

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