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Mouse Anti-LARP7 Recombinant Antibody (CBYJL-1192) (CBMAB-L0696-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to La Ribonucleoprotein Domain Family Member 7
(LARP7). The antibody can be used for immunoassay techniques, such as WB, IP, IF, ELISA.
See all LARP7 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
CBYJL-1192
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
La ribonucleoprotein domain family, member 7
Introduction
LARP7 is a protein which is found in the 7SK snRNP (small nuclear ribonucleoprotein). This snRNP complex inhibits a cyclin-dependent kinase, positive transcription elongation factor b, which is required for paused RNA polymerase II at a promoter to begin transcription elongation. A pseudogene of this gene is located on chromosome 3. Alternative splicing results in multiple transcript variants. Diseases associated with LARP7 include Alazami Syndrome and Med23. Gene Ontology (GO) annotations related to this gene include nucleic acid binding and RNA binding. An important paralog of this gene is LARP6. LARP7 is a negative transcriptional regulator of polymerase II genes, acting by means of the 7SK RNP system.
Entrez Gene ID
Human51574
Mouse28036
Rat686883
UniProt ID
HumanQ4G0J3
MouseQ05CL8
RatQ5XI01
Alternative Names
ALAZS; HDCMA18P; PIP7S
Function
RNA-binding protein that specifically binds distinct small nuclear RNA (snRNAs) and regulates their processing and function (PubMed:18249148, PubMed:32017898).
Specifically binds the 7SK snRNA (7SK RNA) and acts as a core component of the 7SK ribonucleoprotein (RNP) complex, thereby acting as a negative regulator of transcription elongation by RNA polymerase II (PubMed:18249148, PubMed:18483487).
The 7SK RNP complex sequesters the positive transcription elongation factor b (P-TEFb) in a large inactive 7SK RNP complex preventing RNA polymerase II phosphorylation and subsequent transcriptional elongation (PubMed:18249148, PubMed:18483487).
The 7SK RNP complex also promotes snRNA gene transcription by RNA polymerase II via interaction with the little elongation complex (LEC) (PubMed:28254838).
LARP7 specifically binds to the highly conserved 3'-terminal U-rich stretch of 7SK RNA; on stimulation, remains associated with 7SK RNA, whereas P-TEFb is released from the complex (PubMed:18483487, PubMed:18281698).
LARP7 also acts as a regulator of mRNA splicing fidelity by promoting U6 snRNA processing (PubMed:32017898).
Specifically binds U6 snRNAs and associates with a subset of box C/D RNP complexes: promotes U6 snRNA 2'-O-methylation by facilitating U6 snRNA loading into box C/D RNP complexes (PubMed:32017898).
U6 snRNA 2'-O-methylation is required for mRNA splicing fidelity (PubMed:32017898).
Binds U6 snRNAs with a 5'-CAGGG-3' sequence motif (PubMed:32017898).
U6 snRNA processing is required for spermatogenesis (By similarity).
Biological Process
Box C/D RNA 3'-end processingManual Assertion Based On ExperimentIMP:UniProtKB
Cell differentiationIEA:UniProtKB-KW
mRNA processingIEA:UniProtKB-KW
Negative regulation of transcription by RNA polymerase IIManual Assertion Based On ExperimentIMP:FlyBase
Negative regulation of transcription elongation from RNA polymerase II promoter1 PublicationIC:FlyBase
Negative regulation of viral transcriptionManual Assertion Based On ExperimentIMP:FlyBase
Positive regulation of protein localization to Cajal bodyManual Assertion Based On ExperimentIDA:UniProtKB
Positive regulation of snRNA transcription by RNA polymerase IIManual Assertion Based On ExperimentIDA:UniProtKB
Regulation of mRNA splicing, via spliceosomeManual Assertion Based On ExperimentIMP:UniProtKB
RNA splicingIEA:UniProtKB-KW
SpermatogenesisISS:UniProtKB
U6 2'-O-snRNA methylationManual Assertion Based On ExperimentIMP:UniProtKB
Cellular Location
Nucleus, nucleoplasm
Involvement in disease
Alazami syndrome (ALAZS):
A syndromic form of primordial dwarfism, a condition characterized by severe growth restriction that has its onset in utero, and results in short stature and undersize. ALAZS patients manifest severe intellectual disability and distinct facial features including malar hypoplasia, deep-set eyes, broad nose, short philtrum, and macrostomia. Some patients have non-specific and inconsistent skeletal findings, for example, scoliosis and mild epiphyseal changes in the proximal phalanges, but no frank dysplasia.

Yang, M., Ma, B., & Liu, X. (2022). MCTS1 promotes laryngeal squamous cell carcinoma cell growth via enhancing LARP7 stability. Clinical and Experimental Pharmacology and Physiology, 49(6), 652-660.

Yan, P., Li, Z., Xiong, J., Geng, Z., Wei, W., Zhang, Y., ... & Zhang, B. (2021). LARP7 ameliorates cellular senescence and aging by allosterically enhancing SIRT1 deacetylase activity. Cell Reports, 37(8).

Yu, H., Zhang, F., Yan, P., Zhang, S., Lou, Y., Geng, Z., ... & Zhang, B. (2021). LARP7 protects against heart failure by enhancing mitochondrial biogenesis. Circulation, 143(20), 2007-2022.

Hasler, D., Meister, G., & Fischer, U. (2021). Stabilize and connect: the role of LARP7 in nuclear non-coding RNA metabolism. RNA biology, 18(2), 290-303.

Wang, X., Li, Z. T., Yan, Y., Lin, P., Tang, W., Hasler, D., ... & Liu, M. F. (2020). LARP7-mediated U6 snRNA modification ensures splicing fidelity and spermatogenesis in mice. Molecular cell, 77(5), 999-1013.

Hasler, D., Meduri, R., Bąk, M., Lehmann, G., Heizinger, L., Wang, X., ... & Meister, G. (2020). The Alazami syndrome-associated protein LARP7 guides U6 small nuclear RNA modification and contributes to splicing robustness. Molecular cell, 77(5), 1014-1031.

Mennie, A. K., Moser, B. A., & Nakamura, T. M. (2018). LARP7-like protein Pof8 regulates telomerase assembly and poly (A)+ TERRA expression in fission yeast. Nature Communications, 9(1), 586.

Eichhorn, C. D., Yang, Y., Repeta, L., & Feigon, J. (2018). Structural basis for recognition of human 7SK long noncoding RNA by the La-related protein Larp7. Proceedings of the National Academy of Sciences, 115(28), E6457-E6466.

Collopy, L. C., Ware, T. L., Goncalves, T., í Kongsstovu, S., Yang, Q., Amelina, H., ... & Tomita, K. (2018). LARP7 family proteins have conserved function in telomerase assembly. Nature communications, 9(1), 557.

Dateki, S., Kitajima, T., Kihara, T., Watanabe, S., Yoshiura, K. I., & Moriuchi, H. (2018). Novel compound heterozygous variants in the LARP7 gene in a patient with Alazami syndrome. Human Genome Variation, 5(1), 1-3.

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

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