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Mouse Anti-NUS1 Recombinant Antibody (6C11) (CBMAB-N4043-WJ)

This product is a Mouse antibody that recognizes NUS1. The antibody 6C11 can be used for immunoassay techniques such as: WB .
See all NUS1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
6C11
Antibody Isotype
IgG1
Application
WB

Basic Information

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!]

Format
Liquid
Buffer
PBS, pH 7.3, 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/mL
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
NUS1 Dehydrodolichyl Diphosphate Synthase Subunit
Introduction
This gene encodes a type I single transmembrane domain receptor, which is a subunit of cis-prenyltransferase, and serves as a specific receptor for the neural and cardiovascular regulator Nogo-B. The encoded protein is essential for dolichol synthesis and protein glycosylation. This gene is highly expressed in non-small cell lung carcinomas as well as estrogen receptor-alpha positive breast cancer cells where it promotes epithelial mesenchymal transition. This gene is associated with the poor prognosis of human hepatocellular carcinoma patients. Naturally occurring mutations in this gene cause a congenital disorder of glycosylation and are associated with epilepsy. A knockout of the orthologous gene in mice causes embryonic lethality before day 6.5. Pseudogenes of this gene have been defined on chromosomes 13 and X. [provided by RefSeq, May 2017]
Entrez Gene ID
116150
UniProt ID
Q96E22
Alternative Names
NUS1 Dehydrodolichyl Diphosphate Synthase Subunit; Nogo-B Receptor; Nuclear Undecaprenyl Pyrophosphate Synthase 1 Homolog; Di-Trans,Poly-Cis-Decaprenylcistransferase; C6orf68; NgBR; Nuclear Undecaprenyl Pyrophosphate Synthase 1 Homolog (S. Cerevisiae); Dehydrodolichyl Diphosphate Synthase Complex Subunit NUS1; Transport And Golgi Organization 14 Homolog (Drosophila);
Function
With DHDDS, forms the dehydrodolichyl diphosphate synthase (DDS) complex, an essential component of the dolichol monophosphate (Dol-P) biosynthetic machinery. Both subunits contribute to enzymatic activity, i.e. condensation of multiple copies of isopentenyl pyrophosphate (IPP) to farnesyl pyrophosphate (FPP) to produce dehydrodolichyl diphosphate (Dedol-PP), a precursor of dolichol phosphate which is utilized as a sugar carrier in protein glycosylation in the endoplasmic reticulum (ER) (PubMed:21572394, PubMed:25066056, PubMed:28842490, PubMed:32817466).
Synthesizes long-chain polyprenols, mostly of C95 and C100 chain length (PubMed:32817466).
Regulates the glycosylation and stability of nascent NPC2, thereby promoting trafficking of LDL-derived cholesterol. Acts as a specific receptor for the N-terminus of Nogo-B, a neural and cardiovascular regulator (PubMed:16835300).
Biological Process
AngiogenesisIEA:UniProtKB-KW
Cell differentiationIEA:UniProtKB-KW
Cholesterol homeostasisIEA:Ensembl
Dolichol biosynthetic processManual Assertion Based On ExperimentIMP:MGI
Dolichyl diphosphate biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Positive regulation of blood vessel endothelial cell migrationManual Assertion Based On ExperimentIMP:BHF-UCL
Positive regulation of nitric-oxide synthase activityManual Assertion Based On ExperimentIMP:BHF-UCL
Protein glycosylationManual Assertion Based On ExperimentIBA:GO_Central
Protein mannosylationIEA:Ensembl
Regulation of intracellular cholesterol transportManual Assertion Based On ExperimentIGI:MGI
Vascular endothelial growth factor signaling pathwayManual Assertion Based On ExperimentIMP:BHF-UCL
Cellular Location
Endoplasmic reticulum membrane
Colocalizes with Nogo-B during VEGF and wound healing angiogenesis.
Involvement in disease
Congenital disorder of glycosylation 1AA (CDG1AA):
A form of congenital disorder of glycosylation, a multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. CDG1AA inheritance is autosomal recessive.
Intellectual developmental disorder, autosomal dominant 55, with seizures (MRD55):
A form of mental retardation, a disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. MRD55 patients suffer from seizures appearing during the first years of life.

Williams, L. J., Waller, S., Qiu, J., Innes, E., Elserafy, N., Procopis, P., ... & Fung, V. S. (2024). DHDDS and NUS1: A Converging Pathway and Common Phenotype. Movement Disorders Clinical Practice, 11(1), 76-85.

Ji, C., Zhao, J., Zhang, J., & Wang, K. (2023). Novel NUS1 variant in a Chinese patient with progressive myoclonus epilepsy: a case report and systematic review. Neurological Sciences, 44(10), 3495-3498.

Liu, W., Ma, C., Xu, H., Wang, L., Xu, W., Zhang, H., ... & Wang, T. (2022). miR-184-5p inhibits cell proliferation, invasion and predicts prognosis of clear cell renal cell carcinoma by targeting NUS1 dehydrodolichyl diphosphate synthase subunit: Results from large-scale comprehensive identification and validation. Journal of Cancer, 13(5), 1398.

Riboldi, G. M., Monfrini, E., Stahl, C., & Frucht, S. J. (2022). NUS1 and epilepsy-myoclonus-ataxia syndrome: an under-recognized entity?. Tremor and Other Hyperkinetic Movements, 12.

Zhang, P., Cui, D., Liao, P., Yuan, X., Yang, N., Zhen, Y., ... & Huang, Q. (2021). Case report: clinical features of a Chinese boy with epileptic seizures and intellectual disabilities who carries a truncated NUS1 variant. Frontiers in Pediatrics, 9, 725231.

Den, K., Kudo, Y., Kato, M., Watanabe, K., Doi, H., Tanaka, F., ... & Matsumoto, N. (2019). Recurrent NUS1 canonical splice donor site mutation in two unrelated individuals with epilepsy, myoclonus, ataxia and scoliosis-a case report. BMC neurology, 19, 1-7.

Prakash, N., Zadikoff, C., Kinsley, L., Lubbe, S., Mencacci, N., & Krainc, D. (2019). Novel Mutation of NUS1 Gene Presenting With Developmental and Epileptic Encephalopathy and Movement Disorders (P4. 8-051).

Ma, J., Ko, T. P., Yu, X., Zhang, L., Ma, L., Zhai, C., ... & Chen, C. C. (2019). Structural insights to heterodimeric cis-prenyltransferases through yeast dehydrodolichyl diphosphate synthase subunit Nus1. Biochemical and biophysical research communications, 515(4), 621-626.

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

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