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Principaux documents

MABN52

Sigma-Aldrich

Anti-REP-1 Antibody, clone 2F1

clone 2F1, from mouse

Synonyme(s) :

choroideremia (Rab escort protein 1), choroideremia, Choroideraemia protein, Rab escort protein 1, Rab geranylgeranyltransferase component A, TCD protein

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About This Item

Code UNSPSC :
12352203
eCl@ss :
32160702
Nomenclature NACRES :
NA.41

Source biologique

mouse

Niveau de qualité

100

Forme d'anticorps

purified immunoglobulin

Type de produit anticorps

primary antibodies

Clone

2F1, monoclonal

Espèces réactives

human

Technique(s)

immunocytochemistry: suitable
immunohistochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable

Isotype

IgG1κ

Numéro d'accès NCBI

NP_000381

Numéro d'accès UniProt

P24386

Conditions d'expédition

wet ice

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... CHM(1121)

Description générale

’ Rab escort protein (REP)-1 is a 653 amino acid protein belonging to the Rab GDI family of proteins. REP-1 binds to newly assembled unprenylated RAB proteins, escorts them to the Rab GGTases, and remains attached during and after the geranylgeranyl transfer reaction. REP proteins are composed of two conserved domains connected by a 150 amino acid insert. The multisheet domain I acts to assemble a Rab-binding platform. α Defects in the expression of REP-1 cause truncation or absence of the protein, and result in a disease called choroideremia (CHM), an X-linked blindness characterized by a progressive dystrophy of the retinal pigment epithelium, retina, and the choroid.

Spécificité

This antibody recognizes REP-1.

Immunogène

Recombinant protein corresponding to human REP-1.

Application

Analyse par immunohistochimie : A previous lot of MABN52 was successfully used in IH, as reported by an independent laboratory (MacDonald, I.M., et al. (2005). Invest Ophthalmol Vis Sci. 46:E-Abstract 540).
Anti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH.

Qualité



µµ

Description de la cible

Poids réel (observé) : env. 83 kDa

Forme physique

Format : Produit purifié

Autres remarques

Concentration : pour connaître la concentration spécifique du lot, voir le certificat d'analyse.

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Code de la classe de stockage

12 - Non Combustible Liquids

Classe de danger pour l'eau (WGK)

WGK 1

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

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Retrouvez la documentation relative aux produits que vous avez récemment achetés dans la Bibliothèque de documents.

Consulter la Bibliothèque de documents

Lewis E Fry et al.
Translational vision science & technology, 10(8), 12-12 (2021-07-14)
Choroideremia results from the deficiency of Rab Escort Protein 1 (REP1), encoded by CHM, involved in the prenylation of Rab GTPases. Here, we investigate whether the transcription and expression of other genes involved in the prenylation of Rab proteins correlates
Nicolas Cereso et al.
Molecular therapy. Methods & clinical development, 1, 14011-14011 (2014-01-01)
Inherited retinal dystrophies (IRDs) comprise a large group of genetically and clinically heterogeneous diseases that lead to progressive vision loss, for which a paucity of disease-mimicking animal models renders preclinical studies difficult. We sought to develop pertinent human cellular IRD
Maria I Patrício et al.
Molecular therapy. Methods & clinical development, 17, 99-106 (2020-01-01)
Recent advances in recombinant adeno-associated virus (rAAV) gene therapy for choroideremia show gene replacement to be a promising approach. It is, however, well known that contact of vector solution with plastic materials in the surgical device may result in non-specific
Nejla Erkilic et al.
Cells, 8(9) (2019-09-14)
Induced pluripotent stem cells (iPSCs) have revolutionized the study of human diseases as they can renew indefinitely, undergo multi-lineage differentiation, and generate disease-specific models. However, the difficulty of working with iPSCs is that they are prone to genetic instability. Furthermore
Maria I Patrício et al.
Molecular therapy. Methods & clinical development, 9, 288-295 (2018-05-01)
Choroideremia (CHM) is a rare, X-linked recessive retinal dystrophy caused by mutations in the CHM gene. CHM is ubiquitously expressed in human cells and encodes Rab escort protein 1 (REP1). REP1 plays a key role in intracellular trafficking through the
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