MABN52
Anti-REP-1 Antibody, clone 2F1
clone 2F1, from mouse
别名:
choroideremia (Rab escort protein 1), choroideremia, Choroideraemia protein, Rab escort protein 1, Rab geranylgeranyltransferase component A, TCD protein
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关于此项目
UNSPSC Code:
12352203
NACRES:
NA.41
eCl@ss:
32160702
Conjugate:
unconjugated
Clone:
2F1, monoclonal
Application:
ICC, IHC, IP, WB
Citations:
6
biological source
mouse
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
2F1, monoclonal
species reactivity
human
technique(s)
immunocytochemistry: suitable, immunohistochemistry: suitable, immunoprecipitation (IP): suitable, western blot: suitable
isotype
IgG1κ
NCBI accession no.
UniProt accession no.
shipped in
wet ice
target post-translational modification
unmodified
Quality Level
Gene Information
human ... CHM(1121)
General description
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.
~83 kDa observed
Immunogen
Recombinant protein corresponding to human REP-1.
Application
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).
Biochem/physiol Actions
This antibody recognizes REP-1.
Physical form
Format: Purified
Analysis Note
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.
Western Blot Analysis: 0.5 µg/mL of this antibody detected REP-1 on 10 µg of 293T cell lysate.
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
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存储类别
12 - Non Combustible Liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
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
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
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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