Search :
Sign in or Register  
Welcome Sign in or Don't have an account?Register

Mouse Anti-LZTR1 Recombinant Antibody (CBYJL-2289) (CBMAB-L2574-YJ)


See all LZTR1 antibodies
Online Inquiry

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
CBYJL-2289
Antibody Isotype
IgG
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
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.

Target

Full Name
LZTR1
Introduction
LZTR1 is a member of the BTB-kelch superfamily. Initially described as a putative transcriptional regulator based on weak homology to members of the basic leucine zipper-like family, the encoded protein subsequently has been shown to localize exclusively to the Golgi network where it may help stabilize the Gogli complex. Deletion of this gene may be associated with DiGeorge syndrome.
Entrez Gene ID
Human8216
Mouse66863
Rat360745
UniProt ID
HumanQ8N653
MouseQ9CQ33
RatB5DFI4
Alternative Names
NS10; BTBD29; LZTR-1; SWNTS2; leucine-zipper-like transcriptional regulator 1
Function
Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates ubiquitination of Ras (K-Ras/KRAS, N-Ras/NRAS and H-Ras/HRAS) (PubMed:30442762, PubMed:30442766, PubMed:30481304).
Is a negative regulator of RAS-MAPK signaling that acts by controlling Ras levels and decreasing Ras association with membranes (PubMed:30442762, PubMed:30442766, PubMed:30481304).
Biological Process
Negative regulation of Ras protein signal transductionManual Assertion Based On ExperimentIDA:UniProtKB
Protein ubiquitinationManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Endomembrane system
Recycling endosome
Golgi apparatus
Involvement in disease
Glioma (GLM):
Gliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes.
Schwannomatosis 2 (SWNTS2):
A cancer predisposition syndrome in which patients develop multiple non-vestibular schwannomas, benign neoplasms that arise from Schwann cells of the cranial, peripheral, and autonomic nerves.
Noonan syndrome 10 (NS10):
A form of Noonan syndrome, a disease characterized by short stature, facial dysmorphic features such as hypertelorism, a downward eyeslant and low-set posteriorly rotated ears, and a high incidence of congenital heart defects and hypertrophic cardiomyopathy. Other features can include a short neck with webbing or redundancy of skin, deafness, motor delay, variable intellectual deficits, multiple skeletal defects, cryptorchidism, and bleeding diathesis. Individuals with Noonan syndrome are at risk of juvenile myelomonocytic leukemia, a myeloproliferative disorder characterized by excessive production of myelomonocytic cells. NS10 inheritance is autosomal dominant.
Noonan syndrome 2 (NS2):
A form of Noonan syndrome, a disease characterized by short stature, facial dysmorphic features such as hypertelorism, a downward eyeslant and low-set posteriorly rotated ears, and a high incidence of congenital heart defects and hypertrophic cardiomyopathy. Other features can include a short neck with webbing or redundancy of skin, deafness, motor delay, variable intellectual deficits, multiple skeletal defects, cryptorchidism, and bleeding diathesis. Individuals with Noonan syndrome are at risk of juvenile myelomonocytic leukemia, a myeloproliferative disorder characterized by excessive production of myelomonocytic cells. NS2 inheritance is autosomal recessive.
PTM
Phosphorylated on tyrosine upon induction of apoptosis, leading to its degradation by the proteasome.
More Infomation

Abe, T., Kanno, S. I., Niihori, T., Terao, M., Takada, S., & Aoki, Y. (2023). LZTR1 deficiency exerts high metastatic potential by enhancing sensitivity to EMT induction and controlling KLHL12-mediated collagen secretion. Cell Death & Disease, 14(8), 556.

Farncombe, K. M., Thain, E., Barnett-Tapia, C., Sadeghian, H., & Kim, R. H. (2022). LZTR1 molecular genetic overlap with clinical implications for Noonan syndrome and schwannomatosis. BMC Medical Genomics, 15(1), 160.

Cuevas-Navarro, A., Rodriguez-Muñoz, L., Grego-Bessa, J., Cheng, A., Rauen, K. A., Urisman, A., ... & Castel, P. (2022). Cross-species analysis of LZTR1 loss-of-function mutants demonstrates dependency to RIT1 orthologs. Elife, 11, e76495.

Zhang, H., Cao, X., Wang, J., Li, Q., Zhao, Y., & Jin, X. (2021). LZTR1: A promising adaptor of the CUL3 family. Oncology Letters, 22(1), 1-10.

Uludağ Alkaya, D., Lissewski, C., Yeşil, G., Zenker, M., & Tüysüz, B. (2021). Expanding the clinical phenotype of RASopathies in 38 Turkish patients, including the rare LZTR1, RAF1, RIT1 variants, and large deletion in NF1. American Journal of Medical Genetics Part A, 185(12), 3623-3633.

Abe, T., Umeki, I., Kanno, S. I., Inoue, S. I., Niihori, T., & Aoki, Y. (2020). LZTR1 facilitates polyubiquitination and degradation of RAS-GTPases. Cell Death & Differentiation, 27(3), 1023-1035.

Song, J., Guo, R., Nie, H., Chai, L., & Mo, R. (2020). Effect and Mechanism of Targeting Leucine Zipper Like Transcription Regulator 1 (LZTR1) Gene in Hepatic Cellular Cancer Cells. Journal of Biomaterials and Tissue Engineering, 10(10), 1490-1495.

Sewduth, R. N., Pandolfi, S., Steklov, M., Sheryazdanova, A., Zhao, P., Criem, N., ... & Sablina, A. A. (2020). The Noonan syndrome gene Lztr1 controls cardiovascular function by regulating vesicular trafficking. Circulation research, 126(10), 1379-1393.

Umeki, I., Niihori, T., Abe, T., Kanno, S. I., Okamoto, N., Mizuno, S., ... & Aoki, Y. (2019). Delineation of LZTR1 mutation-positive patients with Noonan syndrome and identification of LZTR1 binding to RAF1–PPP1CB complexes. Human Genetics, 138, 21-35.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-LZTR1 Recombinant Antibody (CBYJL-2289)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Learn more

Documents

Request bulk or custom quoteOnline Inquiry
Second antibody and isotype control

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Online Inquiry