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Key Documents

MABN52

Sigma-Aldrich

Anti-REP-1 Antibody, clone 2F1

clone 2F1, from mouse

Sinónimos:

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

Código UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41

origen biológico

mouse

Nivel de calidad

100

forma del anticuerpo

purified immunoglobulin

tipo de anticuerpo

primary antibodies

clon

2F1, monoclonal

reactividad de especies

human

técnicas

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

isotipo

IgG1κ

Nº de acceso NCBI

NP_000381

Nº de acceso UniProt

P24386

Condiciones de envío

wet ice

modificación del objetivo postraduccional

unmodified

Información sobre el gen

human ... CHM(1121)

Descripción general

Rab proteins are low molecular weight, ras-related GTPases that bind to a cell membrane’s cytoplasmic surface and function to regulate protein trafficking in both endocytic and secretory pathways. 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. A globular, smaller, α-helical domain II, is thought to participate in membrane-protein interaction. 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.

Especificidad

This antibody recognizes REP-1.

Inmunógeno

Recombinant protein corresponding to human REP-1.

Aplicación

Anti-REP-1 Antibody, clone 2F1 is an antibody against REP-1 for use in WB, IP, IC, IH.
Immunohistochemistry Analysis: 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).

Calidad

Evaluated by Western Blot in 293T cell lysate.

Western Blot Analysis: 0.5 µg/mL of this antibody detected REP-1 on 10 µg of 293T cell lysate.

Descripción de destino

~83 kDa observed

Forma física

Format: Purified

Otras notas

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

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Código de clase de almacenamiento

12 - Non Combustible Liquids

Clase de riesgo para el agua (WGK)

WGK 1

Punto de inflamabilidad (°F)

Not applicable

Punto de inflamabilidad (°C)

Not applicable

Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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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
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

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