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Mouse Anti-Lrpprc Recombinant Antibody (CBYJL-2484) (CBMAB-L2869-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Leucine-Rich Ppr-Motif Containing (Lrpprc). The antibody can be used for immunoassay techniques, such as WB, IP, IF, ELISA.
See all Lrpprc antibodies

Summary

Host Animal
Mouse
Specificity
Mouse
Clone
CBYJL-2484
Antibody Isotype
IgG
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse
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
leucine-rich PPR-motif containing
Introduction
LRPPRC is a leucine-rich protein that has multiple pentatricopeptide repeats (PPR). The precise role of this protein is unknown but studies suggest it may play a role in cytoskeletal organization, vesicular transport, or in transcriptional regulation of both nuclear and mitochondrial genes. The protein localizes primarily to mitochondria and is predicted to have an N-terminal mitochondrial targeting sequence. Mutations in this gene are associated with the French-Canadian type of Leigh syndrome.
Entrez Gene ID
72416
UniProt ID
Q6PB66
Alternative Names
Lsfc; Gp130; C76645; Lrp130; 3110001K13Rik; leucine-rich PPR motif-containing protein, mitochondrial; 130 kDa leucine-rich protein; leucine rich protein LRP130
Function
May play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation which are possibly associated with nuclear mRNA export. May bind mature mRNA in the nucleus outer membrane. In mitochondria binds to poly(A) mRNA. Plays a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. May be involved in transcription regulation. Cooperates with PPARGC1A to regulate certain mitochondrially encoded genes and gluconeogenic genes and may regulate docking of PPARGC1A to transcription factors. Seems to be involved in the transcription regulation of the multidrug-related genes MDR1 and MVP. Part of a nuclear factor that binds to the invMED1 element of MDR1 and MVP gene promoters. Binds single-stranded DNA (By similarity).
Biological Process
Mitochondrion transport along microtubuleManual Assertion Based On ExperimentTAS:HGNC-UCL
mRNA transportIEA:UniProtKB-KW
Negative regulation of mitochondrial RNA catabolic processIEA:Ensembl
Regulation of mitochondrial translationIEA:Ensembl
Cellular Location
Mitochondrion
Nucleus, nucleoplasm
Nucleus inner membrane
Nucleus outer membrane
Seems to be predominantly mitochondrial.
Involvement in disease
Mitochondrial complex IV deficiency, nuclear type 5 (MC4DN5):
An autosomal recessive, severe mitochondrial disease with multisystemic manifestations and early onset. Clinical features include delayed psychomotor development, impaired intellectual development with speech delay, mild dysmorphic facial features, hypotonia, ataxia, and seizures. Brain imaging shows bilaterally symmetrical necrotic lesions in subcortical brain regions. Mortality is high, due to episodes of severe metabolic acidosis and coma.

Zhao, Z., Sun, Y., Tang, J., Yang, Y., & Xu, X. (2023). LRPPRC regulates malignant behaviors, protects mitochondrial homeostasis, mitochondrial function in osteosarcoma and derived cancer stem-like cells. BMC cancer, 23(1), 935.

Zhou, W., Wang, W., Liang, Y., Jiang, R., Qiu, F., Shao, X., ... & Fang, X. (2023). The RNA-binding protein LRPPRC promotes resistance to CDK4/6 inhibition in lung cancer. Nature Communications, 14(1), 4212.

Yang, Y., Yuan, H., Zhao, L., Guo, S., Hu, S., Tian, M., ... & Song, Y. (2022). Targeting the miR-34a/LRPPRC/MDR1 axis collapse the chemoresistance in P53 inactive colorectal cancer. Cell Death & Differentiation, 29(11), 2177-2189.

Wei, W. S., Wang, N., Deng, M. H., Dong, P., Liu, J. Y., Xiang, Z., ... & Yu, C. P. (2021). LRPPRC regulates redox homeostasis via the circANKHD1/FOXM1 axis to enhance bladder urothelial carcinoma tumorigenesis. Redox Biology, 48, 102201.

Liu, J. Y., Chen, Y. J., Feng, H. H., Chen, Z. L., Wang, Y. L., Yang, J. E., & Zhuang, S. M. (2021). LncRNA SNHG17 interacts with LRPPRC to stabilize c-Myc protein and promote G1/S transition and cell proliferation. Cell Death & Disease, 12(11), 970.

Song, K., Li, B., Chen, Y. Y., Wang, H., Liu, K. C., Tan, W., & Zou, J. (2021). LRPPRC regulates metastasis and glycolysis by modulating autophagy and the ROS/HIF1-α pathway in retinoblastoma. Molecular Therapy-Oncolytics, 22, 582-591.

Bennett Jr, J. P., & Keeney, P. M. (2020). Alzheimer’s and Parkinson’s brain tissues have reduced expression of genes for mtDNA OXPHOS Proteins, mitobiogenesis regulator PGC-1α protein and mtRNA stabilizing protein LRPPRC (LRP130). Mitochondrion, 53, 154-157.

Li, W., Dai, Y., Shi, B., Yue, F., Zou, J., Xu, G., ... & Liu, L. (2020). LRPPRC sustains Yap-P27-mediated cell ploidy and P62-HDAC6-mediated autophagy maturation and suppresses genome instability and hepatocellular carcinomas. Oncogene, 39(19), 3879-3892.

Refolo, G., Ciccosanti, F., Di Rienzo, M., Basulto Perdomo, A., Romani, M., Alonzi, T., ... & Fimia, G. M. (2019). Negative regulation of mitochondrial antiviral signaling protein–mediated antiviral signaling by the mitochondrial protein LRPPRC during Hepatitis C virus infection. Hepatology, 69(1), 34-50.

Cui, J., Wang, L., Ren, X., Zhang, Y., & Zhang, H. (2019). LRPPRC: a multifunctional protein involved in energy metabolism and human disease. Frontiers in Physiology, 10, 595.

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

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