Mouse Anti-LRPPRC Recombinant Antibody (CBYJL-1997) (CBMAB-L2091-YJ)

Name: Mouse Anti-LRPPRC Recombinant Antibody (CBYJL-1997)
SKU: CBMAB-L2091-YJ
Rating: 5 (1 reviews)

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Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

CBYJL-1997

Application

WB, IP, IF, ELISA

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.

More Infomation

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

Human	10128
Mouse	72416
Rat	313867

UniProt ID

Human	P42704
Mouse	Q6PB66
Rat	Q5SGE0

Alternative Names

LSFC; GP130; LRP130; CLONE-23970; leucine-rich PPR motif-containing protein, mitochondrial; 130 kDa leucine-rich protein; LRP 130; leucine-rich PPR-motif containing; mitochondrial leucine-rich PPR motif-containing protein

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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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)
Proteogenomic
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Isotype control

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

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