LDLR Antibody

Code CSB-PA09949A0Rb
Size US$166
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  • Immunofluorescent analysis of HepG2 cells using CSB-PA09949A0Rb at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) LDLR Polyclonal antibody
Uniprot No.
Target Names
LDLR
Alternative Names
FH antibody; FHC antibody; LDL R antibody; LDL receptor antibody; LDLCQ2 antibody; Ldlr antibody; LDLR_HUMAN antibody; Low Density Lipoprotein Receptor antibody; Low density lipoprotein receptor class A domain containing protein 3 antibody; Low density lipoprotein receptor familial hypercholesterolemia antibody; Low-density lipoprotein receptor antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Low-density lipoprotein receptor protein (49-244AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The LDLR Antibody (Product code: CSB-PA09949A0Rb) is Non-conjugated. For LDLR Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA09949B0Rb LDLR Antibody, HRP conjugated ELISA
FITC CSB-PA09949C0Rb LDLR Antibody, FITC conjugated
Biotin CSB-PA09949D0Rb LDLR Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, IF
Recommended Dilution
Application Recommended Dilution
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

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.
Gene References into Functions
  1. HepG2 cell lines transfected with siRNA directed to PCSK9 were challenged with Hcy, homocysteine thiolactone (HTL), testosterone, 5alpha-dihydroxytestosterone (5alpha-DHT), or estradiol for 24h, leading to an overt expression of PCSK9 and down-regulated expression of LDLR. PMID: 29660344
  2. A randomized trial and novel SPR technique identifies altered lipoprotein-LDL receptor binding as a mechanism underlying elevated LDL-cholesterol in APOE4s PMID: 28276521
  3. Authors performed an analysis of public databases and literature for every variant published associated with FH, in the genes LDLR, APOB, and PCSK9. PMID: 29261184
  4. In this review we present a broad spectrum of functionally characterized missense LDLr variants identified in patients with Familial hypercholesterolemia (FH), which is mandatory for a definite diagnosis of FH. PMID: 29874871
  5. The frequency of known mutations in the LDLR gene in this cohort of patients was markedly low compared to frequencies reported in other populations. PMID: 29720182
  6. This study adds 9 novel variations and 11 recurrent variations to the spectrum of LDLR gene mutations in Indian population. The in silico analysis for all the variations detected in this study were done to predict the probabilistic effect in pathogenicity of Familial Hypercholesterolemia. PMID: 29269200
  7. Data suggest maternal glycemic response during pregnancy is associated with lower DNA methylation of 4 CpG sites within PDE4B gene in placenta (collected after normal-weight term birth); 3 additional CpG sites are differentially methylated relative to maternal glucose response within TNFRSF1B, LDLR, and BLM genes. (PDE4B = phosphodiesterase-4B; TNFRSF1B = TNF receptor superfamily member-1B; BLM = Bloom syndrome protein) PMID: 29752424
  8. Vesicular stomatitis virus G protein complex with two distinct cysteine-rich domains (CR2 and CR3) of LDL-R PMID: 29531262
  9. Report familial hypercholesterolemia patients with multiple mutations at the LDLR gene presenting with more severe phenotype than single mutants. PMID: 28645073
  10. LDLr in the activated PSFs may become a novel target receptor for controlled drug delivery. PMID: 28686975
  11. Systematic mutation of the AREs (ARE1-3) in the LDLR 3'UTR and expression of each mutant coupled to a luciferase reporter in Huh7 cells demonstrated that ARE1 is required for rapid LDLR mRNA decay and 5-AzaC-induced mRNA stabilization via the IRE1alpha-EGFR-ERK1/2 signaling cascade. PMID: 29208426
  12. The genotype-risk associations were examined between LDLR (rs885765, rs688, rs5925, rs55903358, rs5742911) and obesity-related phenotypes and other comorbidities in Sucre, Venezuela. The association between LDLR rs5742911 ancestral genotype A/A and high risk condition related to HDL-cholesterol was the only one found to be significant:(A/A: 41.50+/-14.81 mg/dL; A/G: 45.00+/-12.07 mg/dL; G/G: 47.17+/-9.43 mg/dL). PMID: 27622441
  13. Heparan sulfate proteoglycans binding is required for PCSK9-induced LDLR degradation. PMID: 28894089
  14. membrane LDLR was reduced and lost the ability to take up LDL. Our data also expand the spectrum of known LDLR mutations PMID: 29228028
  15. Liposomes modified with both apolipoproteins A-I and E were internalized in HepG2 cells in FBS-depleted culture medium at the same levels as unmodified liposomes in FBS-containing culture medium, which indicates that apolipoproteins A-I and E were the major serum components involved in liposomal binding to SR-B1 or LDLR (or both). PMID: 28888368
  16. These findings suggest that LDLR rs2738464 may affect the affinity of miR-330 binding to the LDLR 3'-UTR, thus regulating LDLR expression and contributing to clear cell renal cell carcinoma risk PMID: 29029037
  17. the p.(Gly20Arg) change in the LDL receptor, previously described as disease causing, has no detrimental effect on protein expression or LDL particle binding PMID: 27175606
  18. Twenty mutations including synonymous, missense, and intronic mutations were identified in the LDLR coding region of 32 Brazilian patients with familial hypercholesterolemia. PMID: 28873201
  19. Results indicate the importance of the LDL receptor (LDLR) in the growth of triple-negative and HER2-overexpressing breast cancers in the setting of elevated circulating LDL cholesterol (LDL-C). PMID: 28759039
  20. Identify LDLR, APOB and PCSK9 novel mutations causing familial hypercholesterolemia in the central south region of China. PMID: 28235710
  21. This study updates the LDLR variant database and identifies a number of reported variants of unknown significance where additional family and in vitro studies will be required to confirm or refute their pathogenicity. PMID: 27821657
  22. Data indicate that proteasomal degradation, lysosomal degradation, autophagy or ectodomain cleavage were not the underlying mechanism for degradation of these mutant LDLRs. PMID: 28334946
  23. PCSK9 inhibits lipoprotein(a) clearance through the LDLR. PMID: 28750079
  24. 4 siblings found to be compound heterozygotes for 2 LDLR gene mutations but showing a different phenotype severity PMID: 27578127
  25. LDLR is a relevant receptor for CNS drug delivery via receptor-mediated transcytosis and that the peptide vectors we developed have the potential to transport drugs PMID: 28108572
  26. Higher Gleason grade was associated with lower LDLR expression, lower SOAT1 and higher SQLE expression. Besides high SQLE expression, cancers that became lethal despite primary treatment were characterized by low LDLR expression (odds ratio for highest versus lowest quintile, 0.37; 95% CI 0.18-0.76) and by low SOAT1 expression (odds ratio, 0.41; 95% CI 0.21-0.83). PMID: 28595267
  27. LDLR associated with Familial Hypercholesterolemia and Polygenic Hypercholesterolemia in patients with Acute Coronary Syndrome , age /=160 mg/dl. PMID: 28958330
  28. Chinese W483X mutation in the low-density lipoprotein-receptor gene in young patients with homozygous familial hypercholesterolemia PMID: 27206941
  29. Proprotein convertase subtilisin/kexin 9 V4I variant with LDLR mutations modifies the phenotype of familial hypercholesterolemia PMID: 27206942
  30. both LDLR rs6511720 and rs57217136 are functional variants; both these minor alleles create enhancer-binding protein sites for transcription factors and may contribute to increased LDLR expression, which is consequently associated with reduced LDL-C levels and 12% lower coronary heart disease risk PMID: 27973560
  31. Using assays that measured conformational change, acid-dependent lipoprotein release, LDLR recycling, and net lipoprotein uptake, we show that H635 plays important roles in acid-dependent conformational change and lipoprotein release, while H264, H306, and H439 play ancillary roles in the response of the LDLR to acidic pH PMID: 27895090
  32. these studies support that reductions in Lp(a) with PCSK9 inhibition are partly due to increased LDLR-mediated uptake. In most situations, Lp(a) appears to compete poorly with LDL for LDLR binding and internalization, but when LDLR expression is increased with evolocumab, particularly in the setting of low circulating LDL, Lp(a) is reduced. PMID: 27102113
  33. Genetic etiology of familial hypercholesterolemia was confirmed in 103 probands following analysis of the whole LDLR gene in a Slovak population. PMID: 27824480
  34. Heterozygous Familial Hypercholesterolemia patients with the null low-density lipoprotein (LDL) receptor DEL15Kb mutation develop severe Aortic Calcifications in an age- and gene dosage-dependent manner. PMID: 28449836
  35. hepatocytes clear lipopolysaccharides from the circulation via the LDLR. PMID: 27171436
  36. the zymogen form of PCSK9 adopts a structure that is distinct from the processed form and is unable to bind a mimetic peptide based on the EGF-A domain of the LDLr. PMID: 27534510
  37. PCSK9 C-terminal domain (CTD) was found to be essential to induce LDLR degradation both upon its overexpression in cells or via the extracellular pathway. PMID: 27280970
  38. LDLR mutation is associated in children and adolescent with familial hypercholesterolemia. PMID: 28161202
  39. Even though LDLR-R410S and LDLR-WT were similar in levels of cell surface and total receptor and bound equally well to LDL or extracellular PCSK9, the LDLR-R410S was resistant to exogenous PCSK9-mediated degradation in endosomes/lysosomes and showed reduced LDL internalization and degradation relative to LDLR-WT. PMID: 27998977
  40. study provides the first evidence that GPC3 can modulate the PCSK9 extracellular activity as a competitive binding partner to the LDLR in HepG2 cells. PMID: 27758865
  41. ox-LDL play a role in the pathogenesis of AMD by NLRP3 inflammasome activation. Suppression of NLRP3 inflammasome activation could attenuate RPE degeneration and AMD progression. PMID: 27607416
  42. Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) Single Domain Antibodies Are Potent Inhibitors of Low Density Lipoprotein Receptor Degradation. PMID: 27284008
  43. This study demonstrated that IL-2 and IL-10 were related to gene polymorphisms of LDL-R, which might be involved in the development and progress of hypercholesterolemia. PMID: 27121486
  44. Lipoprotein profiles get improved by liver-directed gene transfer of human LDLR gene in hypercholesterolaemia mice. PMID: 27350674
  45. Multiple novel LDLR and ApoB mutations have been identified in a-United Kingdom-based cohort with familial hypercholesterolemia. PMID: 26748104
  46. Mutations in LDLR is associated with coronary artery disease. PMID: 26927322
  47. LDLR A(+)A(+) genotype, ApoB X(+) allele and ApoE E4 allele increased the risk of premature coronary artery disease by 1.8, 2.1 and 12.1 respectively. PMID: 27236033
  48. The TT genotype of rs688 in the LDLR gene was not found to be associated with elevated levels of total cholesterol or LDL-C PMID: 25601895
  49. Report increased intestinal cholesterol absorption and elevated serum cholesterol in families with primary hypercholesterolemia without mutations in LDLR. PMID: 26802983
  50. We have used atomistic simulations to explore the complete SNP mutational space (227 mutants) of the LA5 repeat, the key domain for interacting with LDL that is coded in the exon concentrating the highest number of mutations. PMID: 26755827

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Involvement in disease
Familial hypercholesterolemia (FH)
Subcellular Location
Cell membrane; Single-pass type I membrane protein. Membrane, clathrin-coated pit. Golgi apparatus. Early endosome. Late endosome. Lysosome.
Protein Families
LDLR family
Database Links

HGNC: 6547

OMIM: 143890

KEGG: hsa:3949

STRING: 9606.ENSP00000454071

UniGene: Hs.213289

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