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Rabbit Anti-LDLR Recombinant Antibody (101) (CBMAB-L0988-YJ)


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Summary

Host Animal
Rabbit
Specificity
Human
Clone
101
Antibody Isotype
IgG
Application
ELISA

Basic Information

Immunogen
Recombinant Human LDLR protein.
Specificity
Human
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
LDLR
Introduction
The low density lipoprotein receptor (LDLR) gene family consists of cell surface proteins involved in receptor-mediated endocytosis of specific ligands. Low density lipoprotein (LDL) is normally bound at the cell membrane and taken into the cell ending up in lysosomes where the protein is degraded and the cholesterol is made available for repression of microsomal enzyme 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase, the rate-limiting step in cholesterol synthesis. At the same time, a reciprocal stimulation of cholesterol ester synthesis takes place. Mutations in this gene cause the autosomal dominant disorder, familial hypercholesterolemia. Alternate splicing results in multiple transcript variants.
Entrez Gene ID
3949
UniProt ID
P01130
Alternative Names
FH; FHC; LDLCQ2
Function
Binds LDL, the major cholesterol-carrying lipoprotein of plasma, and transports it into cells by endocytosis. In order to be internalized, the receptor-ligand complexes must first cluster into clathrin-coated pits.
(Microbial infection) Acts as a receptor for hepatitis C virus in hepatocytes, but not through a direct interaction with viral proteins.
(Microbial infection) Acts as a receptor for Vesicular stomatitis virus.
(Microbial infection) In case of HIV-1 infection, may function as a receptor for extracellular Tat in neurons, mediating its internalization in uninfected cells.
Biological Process
Amyloid-beta clearanceISS:ARUK-UCL
Amyloid-beta clearance by cellular catabolic processISS:ARUK-UCL
Artery morphogenesisIEA:Ensembl
Cellular response to fatty acidIEA:Ensembl
Cellular response to low-density lipoprotein particle stimulusManual Assertion Based On ExperimentIMP:BHF-UCL
Cholesterol homeostasisManual Assertion Based On ExperimentIMP:BHF-UCL
Cholesterol importManual Assertion Based On ExperimentIMP:BHF-UCL
Cholesterol metabolic processIEA:UniProtKB-KW
Cholesterol transportManual Assertion Based On ExperimentIMP:HGNC-UCL
EndocytosisManual Assertion Based On ExperimentTAS:ProtInc
High-density lipoprotein particle clearanceIEA:Ensembl
Intestinal cholesterol absorptionManual Assertion Based On ExperimentIMP:HGNC-UCL
Lipid metabolic processManual Assertion Based On ExperimentTAS:ProtInc
Lipoprotein catabolic processIEA:Ensembl
Long-term memoryManual Assertion Based On ExperimentIGI:ARUK-UCL
Low-density lipoprotein particle clearanceManual Assertion Based On ExperimentIMP:BHF-UCL
Negative regulation of amyloid fibril formationISS:ARUK-UCL
Negative regulation of astrocyte activationISS:ARUK-UCL
Negative regulation of gene expressionIEA:Ensembl
Negative regulation of low-density lipoprotein particle clearanceManual Assertion Based On ExperimentIDA:ComplexPortal
Negative regulation of microglial cell activationISS:ARUK-UCL
Negative regulation of protein metabolic processISS:ARUK-UCL
Negative regulation of receptor recyclingManual Assertion Based On ExperimentIDA:ComplexPortal
PhagocytosisISS:ARUK-UCL
Phospholipid transportISS:BHF-UCL
Plasma lipoprotein particle clearanceISS:ARUK-UCL
Positive regulation of gene expressionIEA:Ensembl
Positive regulation of inflammatory responseIEA:Ensembl
Positive regulation of lysosomal protein catabolic processISS:ARUK-UCL
Positive regulation of triglyceride biosynthetic processISS:BHF-UCL
Receptor-mediated endocytosisISS:ARUK-UCL
Receptor-mediated endocytosis involved in cholesterol transportManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of cholesterol metabolic processIEA:Ensembl
Regulation of phosphatidylcholine catabolic processISS:BHF-UCL
Regulation of protein metabolic processManual Assertion Based On ExperimentIGI:ARUK-UCL
Response to caloric restrictionManual Assertion Based On ExperimentIGI:ARUK-UCL
Cellular Location
Cell membrane
Membrane, clathrin-coated pit
Golgi apparatus
Early endosome
Late endosome
Lysosome
Rapidly endocytosed upon ligand binding.
Involvement in disease
Hypercholesterolemia, familial, 1 (FHCL1):
A form of hypercholesterolemia, a disorder of lipoprotein metabolism characterized by elevated serum low-density lipoprotein (LDL) cholesterol levels, which result in excess deposition of cholesterol in tissues and leads to xanthelasma, xanthomas, accelerated atherosclerosis and increased risk of premature coronary heart disease. FHCL1 inheritance is autosomal dominant.
Topology
Extracellular: 22-788
Helical: 789-810
Cytoplasmic: 811-860
PTM
N- and O-glycosylated.
Ubiquitinated by MYLIP leading to degradation.
More Infomation

Calvier, L., Herz, J., & Hansmann, G. (2022). Interplay of low-density lipoprotein receptors, LRPs, and lipoproteins in pulmonary hypertension. Basic to Translational Science, 7(2), 164-180.

Huang, L., Li, H., Ye, Z., Xu, Q., Fu, Q., Sun, W., ... & Yue, J. (2021). Berbamine inhibits Japanese encephalitis virus (JEV) infection by compromising TPRMLs-mediated endolysosomal trafficking of low-density lipoprotein receptor (LDLR). Emerging Microbes & Infections, 10(1), 1257-1271.

Floeth, M., Elges, S., Gerss, J., Schwöppe, C., Kessler, T., Herold, T., ... & Angenendt, L. (2021). Low‐density lipoprotein receptor (LDLR) is an independent adverse prognostic factor in acute myeloid leukaemia. British Journal of Haematology, 192(3), 494-503.

Campion, O., Al Khalifa, T., Langlois, B., Thevenard-Devy, J., Salesse, S., Savary, K., ... & Devy, J. (2020). Contribution of the low-density lipoprotein receptor family to breast cancer progression. Frontiers in Oncology, 10, 882.

Sun, R., Peng, M., Xu, P., Huang, F., Xie, Y., Li, J., ... & Zhu, W. (2020). Low-density lipoprotein receptor (LDLR) regulates NLRP3-mediated neuronal pyroptosis following cerebral ischemia/reperfusion injury. Journal of neuroinflammation, 17(1), 1-17.

Wang, Z., Duan, X., Lv, Y., & Zhao, Y. (2019). Low density lipoprotein receptor (LDLR)-targeted lipid nanoparticles for the delivery of sorafenib and Dihydroartemisinin in liver cancers. Life sciences, 239, 117013.

Banerjee, P., Chan, K. C., Tarabocchia, M., Benito-Vicente, A., Alves, A. C., Uribe, K. B., ... & Martin, C. (2019). Functional analysis of LDLR (low-density lipoprotein receptor) variants in patient lymphocytes to assess the effect of evinacumab in homozygous familial hypercholesterolemia patients with a spectrum of LDLR activity. Arteriosclerosis, Thrombosis, and Vascular Biology, 39(11), 2248-2260.

Burke, A. C., Telford, D. E., Sutherland, B. G., Edwards, J. Y., Sawyez, C. G., Barrett, P. H. R., ... & Huff, M. W. (2018). Bempedoic Acid Lowers Low-Density Lipoprotein Cholesterol and Attenuates Atherosclerosis in Low-Density Lipoprotein Receptor–Deficient (LDLR+/− and LDLR−/−) Yucatan Miniature Pigs. Arteriosclerosis, thrombosis, and vascular biology, 38(5), 1178-1190.

Thedrez, A., Blom, D. J., Ramin-Mangata, S., Blanchard, V., Croyal, M., Chemello, K., ... & Lambert, G. (2018). Homozygous Familial Hypercholesterolemia Patients With Identical Mutations Variably Express the LDLR (Low-Density Lipoprotein Receptor) Implications for the Efficacy of Evolocumab. Arteriosclerosis, thrombosis, and vascular biology, 38(3), 592-598.

Lu, R., Yuan, T., Wang, Y., Zhang, T., Yuan, Y., Wu, D., ... & Cheng, Y. (2018). Spontaneous severe hypercholesterolemia and atherosclerosis lesions in rabbits with deficiency of low-density lipoprotein receptor (LDLR) on exon 7. EBioMedicine, 36, 29-38.

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

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