推薦產品
生物源
mouse
品質等級
抗體表格
purified immunoglobulin
抗體產品種類
primary antibodies
無性繁殖
2F1, monoclonal
物種活性
human
技術
immunocytochemistry: suitable
immunohistochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
同型
IgG1κ
NCBI登錄號
UniProt登錄號
運輸包裝
wet ice
目標翻譯後修改
unmodified
基因資訊
human ... CHM(1121)
一般說明
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.
特異性
This antibody recognizes REP-1.
免疫原
Recombinant protein corresponding to human REP-1.
應用
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).
品質
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.
標靶描述
~83 kDa observed
外觀
Format: Purified
其他說明
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
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儲存類別代碼
12 - Non Combustible Liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
分析證明 (COA)
輸入產品批次/批號來搜索 分析證明 (COA)。在產品’s標籤上找到批次和批號，寫有 ‘Lot’或‘Batch’.。
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
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
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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