EBP Antibody

Datasheet

Code	CSB-PA624016EA01HU
Size	US$166
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Image	Western blot All lanes: EBP antibody at 2µg/ml Lane 1: A549 whole cell lysate Lane 2: 293T whole cell lysate Secondary Goat polyclonal to rabbit IgG at 1/10000 dilution Predicted band size: 27 kDa Observed band size: 27 kDa Immunohistochemistry of paraffin-embedded human breast cancer using CSB-PA624016EA01HU at dilution of 1:100 Immunohistochemistry of paraffin-embedded human lung cancer using CSB-PA624016EA01HU at dilution of 1:100 Immunofluorescent analysis of 293T cells using CSB-PA624016EA01HU at dilution of 1:100 and 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) EBP Polyclonal antibody

Uniprot No.

Q15125

Target Names

EBP

Alternative Names

EBP; 3-beta-hydroxysteroid-Delta(8,Delta(7-isomerase; Cholestenol Delta-isomerase; Delta(8-Delta(7 sterol isomerase; D8-D7 sterol isomerase; Emopamil-binding protein

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase protein (2-230AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The EBP Antibody (Product code: CSB-PA624016EA01HU) is Non-conjugated. For EBP Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA624016EB01HU	EBP Antibody, HRP conjugated	ELISA
FITC	CSB-PA624016EC01HU	EBP Antibody, FITC conjugated
Biotin	CSB-PA624016ED01HU	EBP 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, WB, IHC, IF

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:20-1:200
IF	1:50-1:200

Protocols

ELISA Protocol
Western Blotting(WB) 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.

Customer Reviews and Q&A

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Target Background

Function

Catalyzes the conversion of Delta(8)-sterols to their corresponding Delta(7)-isomers.

Gene References into Functions

This study expands the current phenotypic spectrum of males with hypomorphic EBP mutations and supports to the hypothesis that there exists an X-linked recessive entity independent of CDPX2. PMID: 24700572
Mutation analysis revealed a heterozygous novel missense mutation, c.204G>T (p.W68C), in exon 2. PMID: 24915996
With non-mosaic EBP mutations in males. PMID: 24459067
Report steroidomimetic aminomethyl spiroacetals as novel inhibitors of the enzyme Delta8,7-sterol isomerase in cholesterol biosynthesis. PMID: 24493593
Elastin binding protein and FKBP65 modulate the kinetics of self-assembly of tropoelastin in an in vitro system. PMID: 24106871
Results show a clear relationship between abnormal sterol profile and the EBP gene mutation PMID: 22121851
postzygotic mosaicism on an ichthyosiform skin lesion in the mother of a girl with X-linked dominant chondrodysplasia punctata associated with a novel EBP mutation. PMID: 21931045
Molecular analysis of EBP mutations were made. PMID: 17378690
Emopamil binding protein (EBP)-shRNA sequences were designed and tested for their effectiveness. PMID: 17498944
We found two novel (3G-->T and 419-422delTTCT) and one known mutation in the EBP gene. The strong phenotypic variability in our patients suggests that there is no clear genotype-phenotype correlation. PMID: 17949453
two unrelated Thai girls with chondrodysplasia punctata type 2. Mutation analysis by PCR-sequencing the entire coding region of emopamil binding protein(EBP) successfully revealed two potentially pathogenic, novel mutations, c.616G-->T and c.382delC. PMID: 18573709

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Involvement in disease

Chondrodysplasia punctata 2, X-linked dominant (CDPX2); MEND syndrome (MEND)

Subcellular Location

Endoplasmic reticulum membrane; Multi-pass membrane protein. Nucleus envelope. Cytoplasmic vesicle.

Protein Families

EBP family

Database Links

HGNC: 3133

OMIM: 300205

KEGG: hsa:10682

STRING: 9606.ENSP00000417052

UniGene: Hs.30619

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

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

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