UBR1 Antibody

Datasheet

Code	CSB-PA812883LA01HU
Size	US$166
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Image	IHC image of CSB-PA812883LA01HU diluted at 1:500 and staining in paraffin-embedded human pancreatic tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. Immunofluorescence staining of U251 cells with CSB-PA812883LA01HU at 1:166, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) UBR1 Polyclonal antibody

Uniprot No.

Q8IWV7

Target Names

UBR1

Alternative Names

E3 ubiquitin-protein ligase UBR1 antibody; JBS antibody; N-recognin-1 antibody; ubiquitin protein ligase E3 component n-recognin 1 antibody; Ubiquitin-protein ligase E3-alpha-1 antibody; Ubiquitin-protein ligase E3-alpha-I antibody; UBR1 antibody; UBR1_HUMAN antibody

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human E3 ubiquitin-protein ligase UBR1 protein (722-862AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The UBR1 Antibody (Product code: CSB-PA812883LA01HU) is Non-conjugated. For UBR1 Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA812883LB01HU	UBR1 Antibody, HRP conjugated	ELISA
FITC	CSB-PA812883LC01HU	UBR1 Antibody, FITC conjugated
Biotin	CSB-PA812883LD01HU	UBR1 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, IHC, IF

Recommended Dilution

Application	Recommended Dilution
IHC	1:500-1:1000
IF	1:50-1:200

Protocols

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol

Troubleshooting and FAQs

Antibody 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.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Customer Reviews and Q&A

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Target Background

Function

E3 ubiquitin-protein ligase which is a component of the N-end rule pathway. Recognizes and binds to proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their ubiquitination and subsequent degradation. May be involved in pancreatic homeostasis. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth.

Gene References into Functions

UBR1 mutations of single or multi-exon deletions or duplications account for a substantial proportion of Johanson-Blizzard syndrome. PMID: 29178640
The frequency of any non-synonymous or synonymous variants was not different between the patients with chronic pancreatitis and controls PMID: 27397733
Reduced UBR1 expression affects MGMT turnover and DNA repair in the smokers lungs. PMID: 26183928
For all previously reported and novel UBR1 mutations together with their clinical data, a mutation database has been established at LOVD. PMID: 24599544
Testing the fetus and the affected sibling with recurrent Johanson-Blizzard syndrome revealed a homozygous truncating mutation in UBR1. PMID: 21711208
Results confirmed the relevance of specific missense UBR1 alleles to JBS, and suggested that a residual activity of a missense allele is causally associated with milder variants of JBS. PMID: 21931868
Molecular studies revealed a novel homozygous nonsense mutation in exon 38 of the UBR1 gene, which confirmed the diagnosis of Johanson-Blizzard syndrome. PMID: 20556423
Ubc2/Rad6 ser(120) regulates ubiquitin-dependent N-end rule targeting by E3{alpha}/Ubr1 PMID: 21041297
Deficiency of UBR1 perturbs the pancreas' acinar cells and other organs, presumably owing to metabolic stabilization of specific substrates of the N-end rule pathway. PMID: 16311597
Sustained ERK5 activity and the E3 ligase UBR1 regulate the stability and subcellular localization of c-Fos. PMID: 17018293
Thus we propose that autoISGylation of EFP negatively regulates its ISG15 E3 ligase activity for 14-3-3sigma. PMID: 17222803
These results suggest that Rabring7 is involved in the endocytic trafficking of EGFR through its E3 ligase activity. PMID: 17462600
Thiazolidinediones modulate the expression of beta catenin and other cell cycle proteins by targeting UBR1 independently of PPARG. PMID: 17569795
E3 ubiquitin ligase is an essential downstream component of the RAS signal transduction pathway. PMID: 18089810
study reports on two apparently unrelated girls with Johanson-Blizzard syndrome, in both children caused by a homozygous IVS26+5G>A mutation in the UBR1 gene PMID: 19006206
Case Report: Johanson-Blizzard syndrome with mild phenotypic features confirmed by UBR1 gene testing. PMID: 19058315

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Involvement in disease

Johanson-Blizzard syndrome (JBS)

Subcellular Location

Cytoplasm, cytosol.

Protein Families

UBR1 family

Tissue Specificity

Broadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).

Database Links

HGNC: 16808

OMIM: 243800

KEGG: hsa:197131

STRING: 9606.ENSP00000290650

UniGene: Hs.591121

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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