UBQLN1 Antibody, HRP conjugated
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Description
Introduction to UBQLN1 Antibody, HRP Conjugated
The UBQLN1 Antibody, HRP conjugated (Product Code: CSB-PA892477LB01HU), is a specialized immunological reagent designed for detecting the human UBQLN1 protein in research settings. UBQLN1 (Ubiquilin-1) is a critical regulator of proteostasis, mediating protein degradation via the ubiquitin-proteasome system (UPS) and autophagy pathways . The HRP (horseradish peroxidase) conjugation enhances its utility in enzyme-linked immunosorbent assays (ELISA), enabling sensitive and quantitative detection of UBQLN1 in biological samples.
Key Features
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Applications: ELISA, Western blot (WB), immunohistochemistry (IHC), and immunofluorescence (IF) .
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Conjugate: HRP, optimized for colorimetric or chemiluminescent detection .
| Parameter | Detail |
|---|---|
| Immunogen | Synthetic peptide spanning amino acids 296–326 of human UBQLN1 . |
| Host/Isotype | Rabbit polyclonal IgG . |
| Recommended Dilution | ELISA: 1:20–1:200; IHC: 1:50–1:200; IF: 1:50–1:200 . |
Applications in Research
The UBQLN1 Antibody, HRP conjugated, has been employed in diverse studies to investigate UBQLN1’s role in cellular processes and diseases.
2.1. Proteostasis and Mitochondrial Function
UBQLN1 is implicated in clearing mislocalized mitochondrial proteins and regulating electron transport chain (ETC) components . A study using UBQLN1 antibodies (including ELISA-based detection) demonstrated that UBQLN1 deficiency leads to mitochondrial dysfunction, marked by reduced ETC proteins and increased autophagy markers (e.g., LC3-II) .
2.2. Telomere Regulation and DNA Damage Response
Research has shown that UBQLN1 shuttles ubiquitinated RPA1 from replication forks to prevent telomere shortening . Antibodies targeting UBQLN1 (e.g., CSB-PA892477LB01HU) were used to validate RPA1-UBQLN1 interactions via co-immunoprecipitation and ELISA .
2.3. Amyotrophic Lateral Sclerosis (ALS)
A 2024 study identified lnc-ABCA12-3 as a regulator of UBQLN1 expression in ALS models . While not directly using the HRP-conjugated antibody, the findings underscore the antibody’s potential utility in ALS research .
3.1. Immunoblot Validation
Western blot analysis of SH-SY5Y lysates (35 μg/lane) confirmed specific detection of UBQLN1 (~60 kDa) .
3.2. ELISA Protocol Example
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Coat plates with UBQLN1 protein (0.5–2 μg/mL).
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Incubate with primary antibody (1:100 dilution in PBS-T).
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Detect using HRP-conjugated secondary antibody and TMB substrate .
4.1. Ubiquitin Binding Specificity
Full-length UBQLN1 preferentially binds K63-linked ubiquitin chains, a specificity absent in isolated UBA domains . This property is critical for its role in autophagy and proteasomal degradation .
4.2. ALS Pathogenesis
Knockdown of lnc-ABCA12-3 reduced UBQLN1 expression, linking its dysregulation to ALS progression .
Product Specs
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Lead Time
Generally, we can ship the products within 1-3 business days after receiving your orders. Delivery time may vary depending on the purchase method or location. Please consult your local distributors for specific delivery times.
Synonyms
DA41 antibody; DSK2 antibody; FLJ90054 antibody; hPLIC-1 antibody; hPLIC1 antibody; PLIC-1 antibody; PLIC1 antibody; Protein linking IAP with cytoskeleton 1 antibody; Ubiquilin-1 antibody; Ubiquilin1 antibody; UBQL1_HUMAN antibody; UBQLN1 antibody; UBQN antibody; XDRP1 antibody
Target Names
Uniprot No.
Target Background
Function
UBQLN1 plays a crucial role in regulating various protein degradation mechanisms and pathways, including the ubiquitin-proteasome system (UPS), autophagy, and endoplasmic reticulum-associated protein degradation (ERAD) pathway. It facilitates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding to their polyubiquitin chains through its UBA domain and interacting with the proteasome subunits through its ubiquitin-like domain. UBQLN1 participates in the ERAD pathway by interacting with ER-localized proteins UBXN4, VCP, and HERPUD1, potentially bridging polyubiquitinated ERAD substrates with the proteasome. Isoform 1, isoform 2, and isoform 3 contribute to the unfolded protein response (UPR) by attenuating the induction of UPR-inducible genes (DDTI3/CHOP, HSPA5, and PDIA2) during ER stress. UBQLN1 is involved in the regulation of macroautophagy and autophagosome formation, required for the maturation of autophagy-related protein LC3 from LC3-I to LC3-II, and may facilitate autophagosome-lysosome fusion. It negatively regulates the TICAM1/TRIF-dependent toll-like receptor signaling pathway by reducing TICAM1 abundance through the autophagic pathway. Isoform 1 and isoform 3 are critical for regulating PSEN1 levels by targeting its accumulation to aggresomes, which can then be removed from cells via autophagocytosis. UBQLN1 promotes the ubiquitination and lysosomal degradation of ORAI1, consequently downregulating ORAI1-mediated Ca2+ mobilization. It suppresses the maturation and proteasomal degradation of amyloid beta A4 protein (A4) by stimulating lysine 63 (K63)-linked polyubiquitination, delaying A4 maturation by sequestering it in the Golgi apparatus and preventing its transport to the cell surface for subsequent processing.
Gene References Into Functions
- Thus, UBQLN1 plays an important role in clearing mislocalized mitochondrial proteins upon cell stimulation, and its absence leads to suppression of protein synthesis and cell cycle arrest. PMID: 28933694
- UBQLN1 variant was not associated with the risk of Alzheimer's disease. PMID: 28719358
- The STI and UBA Domains of UBQLN1 Are Critical Determinants of Substrate Interaction and Proteostasis. PMID: 28075048
- Following the loss of UBQLN1 in lung adenocarcinoma cells, there is an accelerated loss of IGF1R. PMID: 29054976
- The evidence of genetic associations has been found for the multivariate response phenotype that involves trans effects modulating expression of genes following heat shock, including HSF1 and UBQLN1. PMID: 27553423
- The expression level of UBQLN1 and prognosis in breast cancer is clarified for the first time and UBQLN1 seems to be a novel molecular marker to predict poor prognosis in breast cancer. PMID: 26406952
- The results suggest that UBQ-8i polymorphism may contribute to Alzheimer's disease susceptibility, but does not synergize with APOEepsilon4 status to increase Alzheimer's disease risk. PMID: 25010605
- These results suggest that the UBQ-8i polymorphism is associated with Alzheimer's disease risk. PMID: 25387430
- found that ZEB1 is required for induction of mesenchymal-like properties following loss of UBQLN1 and ZEB1 is capable of repressing expression of UBQLN1 PMID: 24747970
- High UBQLN1 expression is associated with low radiosensitivity in breast cancer. PMID: 25044403
- Ubiquilin-1 immunoreactivity is concentrated on Hirano bodies and dystrophic neurites in Alzheimer's disease PMID: 23421764
- Human ubiquilin-1 overexpression in transgenic mice increases the lifespan and delays accumulation of Huntingtin aggregates in the R6/2 mouse model of Huntington's disease. PMID: 24475300
- The results demonstrate that in Alzheimer's disease hippocampus, ubiquilin-1 immunoreactivity increases in the neuronal nucleoplasm and is associated with region-specific neurofibrillary changes. PMID: 23869942
- Targeting of Ubqln1 to autophagosomes requires the Ubqln4 UBL domain and the Ubqln1 UBA domain. PMID: 23459205
- data suggest that ubiquilin-1 modulates gamma-secretase-mediated epsilon-site cleavage and thus may play a role in regulating gamma-secretase cleavage of various substrates. PMID: 23663107
- Genetic variants in UBQLN1 are not commonly associated with amyotrophic lateral sclerosis PMID: 22766032
- This study demonistrated that Allele C of polymorphism UBQ-8i of the UBQLN1 gene is not an independent risk factor for mild cognitive impairment or Alzheimer's disease PMID: 22272618
- Ubiquilin-1 was over-expressed following antiproliferative agents treatment of ovarian cancer cells. PMID: 22134777
- ubiquilin-1 chaperone activity is necessary to regulate the production of APP and its fragments and that diminished ubiquilin-1 levels may contribute to AD pathogenesis. PMID: 21852239
- PLIC-1 is a novel inhibitor of the TLR3-Trif antiviral pathway by reducing the abundance of Trif PMID: 21695056
- specific ubiquilin-1 transcript variants can cause PS1 accumulation and aggresome formation PMID: 21143716
- Data show that ubiquilin is degraded during both macroautophagy and during chaperone-mediated autophagy (CMA). PMID: 20529957
- The UBQ-8i polymorphism of the UBQLN1 gene is extremely rare in Taiwan Chinese and unlikely to play a significant role in the risk of AD in Taiwan Chinese. PMID: 20350585
- PLIC1 may be a regulator of HCV RNA replication through interaction with NS5B. Nn Huh7 cells that express an HCV subgenomic replicon, the amounts of both NS5B and the replicon RNA were reduced by overexpression of PLIC1. PMID: 12634373
- Ubiquilin proteins play an important role in regulating PS protein levels in cells. PMID: 15004330
- Ubiquilin-1 plays an active role in the precise regulation of HASH-1 and of other tissue-specific bHLH proteins PMID: 15492808
- Our findings suggest that genetic variants in UBQLN1 on chromosome 9q22 substantially increase the risk of Alzheimer's disease, possibly by influencing alternative splicing of this gene in the brain PMID: 15745979
- ubiquilin-1 limits the availability of unassembled nicotinic acetylcholine receptor subunits in neurons by drawing them to the proteosome, thus regulating nicotine-induced up-regulation PMID: 16091357
- Genetic variation in the UBQLN1 gene has a modest effect on risk, age of onset and disease duration of Alaheimer's disease. PMID: 16302009
- Overexpression of ubiquilin reduces cell death in HeLa cells and primary neurons stably expressing green fluorescent protein-huntingtin fusion protein. PMID: 16461334
- Results suggest that UBQLN1 variants do not increase risk for Alzheimer disease. PMID: 16526030
- Data show that ubiquilin 1 interacts both with presenilin 1 (PS1) holoprotein and heterodimer and that the interaction between PS1 and ubiquilin 1 takes place near the cell surface. PMID: 16815845
- UBQLN1 modulates amyloid precursor protein trafficking and Abeta secretion. PMID: 16945923
- These results indicate a role for PLIC-1 in the protein aggregation-stress pathway, and we propose a novel function for the ubiquitin-like (UBL) domain--by means of UBL-UIM interactions--in transport to aggresomes. PMID: 17082820
- Mutation of two lysine residues in the PS2-loop region suggested that ubiquitination is not required for interaction with ubiquilin-1 and may, in fact, even negatively regulate the interaction. PMID: 17614368
- Our results suggest that it is unlikely that the SNP rs12344615 of the UBQLN1 gene is related to the onset of AD, PD or cognitive function. PMID: 17709205
- Expression of the human Alzheimer's disease-associated variant of UBQLN1 leads to more severe degeneration than does comparable expression of the human wildtype UBQLN1 in Drosophila eye. PMID: 17947293
- The three-dimensional structure of the UBA domain of ubiquilin-1 (UQ1-UBA) free in solution and in complex with ubiquitin, is described. PMID: 18241885
- Risk estimation for AA versus GG genotypes showed that the AA genotype is a weak risk factor for Alzheimer's disease PMID: 18340109
- Plic-1 may play a significant role in regulating the strength of synaptic inhibition by increasing the stability of GABA(A)Rs within the secretory pathway and thereby promoting their insertion into the neuronal plasma membrane. PMID: 18467327
- overexpression UBQLN1 transcript variants TV1-3, but not TV4, exert a protective effect during the unfolded protein response by attenuating CHOP induction and potentially increasing cell viability. PMID: 18953672
- siRNA-mediated UBQLN1 depletion made cells more susceptible to starvation-induced cell death. UBQLN1 regulates cell survival during starvation. PMID: 19148225
Database Links
Subcellular Location
Cytoplasm. Nucleus. Endoplasmic reticulum. Cytoplasmic vesicle, autophagosome. Cell membrane.
Tissue Specificity
Brain (at protein level). Ubiquitous. Highly expressed throughout the brain; detected in neurons and in neuropathological lesions, such as neurofibrillary tangles and Lewy bodies. Highly expressed in heart, placenta, pancreas, lung, liver, skeletal muscle
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