MABE70
抗MBNL1抗体，克隆4A8
clone 4A8, from mouse
别名:
muscleblind-like (Drosophila), muscleblind (Drosophila)-like, muscleblind-like protein 1, Triplet-expansion RNA-binding protein
产品名称
抗MBNL1抗体，克隆4A8, clone 4A8, from mouse
biological source
mouse
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
4A8, monoclonal
species reactivity
human
technique(s)
immunocytochemistry: 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 ... MBNL1(4154)
Analysis Note
通过蛋白质印迹对HEK293细胞裂解物中的抗体进行了评估。
蛋白质印迹分析：用该抗体的1:10,000稀释液在10 µg HEK293细胞裂解液中检测MBNL1。
蛋白质印迹分析：用该抗体的1:10,000稀释液在10 µg HEK293细胞裂解液中检测MBNL1。
Application
免疫细胞化学分析：
代表性批次的1：4稀释液在肌强直性营养不良细胞中检测到MBNL1。 数据由Wolfson Centre for Inherited Neuromuscular Disease的Ian Holt博士提供。
代表性批次的1：4稀释液在肌强直性营养不良细胞中检测到MBNL1。 数据由Wolfson Centre for Inherited Neuromuscular Disease的Ian Holt博士提供。
抗MBNL1抗体，克隆4A8是用于检测MBNL1的小鼠单克隆抗体，也称为肌盲样蛋白1，三重扩增RNA结合蛋白&已在WB、IP、ICC中得到验证。
General description
可观察到约39 kDa的条带
盲肌样蛋白 1 (MBNL1) 是盲肌样蛋白家族成员。MBNL1参与前体mRNA的选择性剪接，并充当特定前体mRNA靶标的剪接激活或抑制剂。此蛋白含有四个C3H1型锌指结构域并与扩展的CUG重复序列RNA结合。在心肌、骨骼肌以及分化成肌细胞中，可观察到高表达水平的MBNL1。目前确知MBNL1参与 Ⅰ型强直性肌营养不良症 (DM1) 的发病机制。对于患有这种肌肉疾病的人来说，可用含有CUG三联体重复扩增的DMPK RNA隔离MBNL1 。
Immunogen
对应于人MBNL1的全长重组蛋白。
Physical form
形式：纯化
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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
Raphael I Benhamou et al.
Cell chemical biology, 27(2), 223-231 (2020-01-26)
Myotonic dystrophy type 2 (DM2) is a genetically defined disease caused by a toxic expanded repeat of r(CCUG) [r(CCUG)exp], harbored in intron 1 of CCHC-type zinc-finger nucleic acid binding protein (CNBP) pre-mRNA. This r(CCUG)exp causes toxicity via a gain-of-function mechanism
Raphael I Benhamou et al.
Journal of medicinal chemistry, 63(14), 7827-7839 (2020-07-14)
RNA repeat expansions are responsible for more than 30 incurable diseases. Among them is myotonic dystrophy type 1 (DM1), the most common form of adult on-set muscular dystrophy. DM1 is caused by an r(CUG) repeat expansion [r(CUG)exp] located in the
Fan Zhang et al.
Human molecular genetics, 26(16), 3056-3068 (2017-05-24)
Myotonic dystrophy Type 1 (DM1) is a rare genetic disease caused by the expansion of CTG trinucleotide repeats ((CTG)exp) in the 3' untranslated region of the DMPK gene. The repeat transcripts sequester the RNA binding protein Muscleblind-like protein 1 (MBNL1)
Alicia J Angelbello et al.
Cell chemical biology, 28(1), 34-45 (2020-11-07)
Many diseases are caused by toxic RNA repeats. Herein, we designed a lead small molecule that binds the structure of the r(CUG) repeat expansion [r(CUG)exp] that causes myotonic dystrophy type 1 (DM1) and Fuchs endothelial corneal dystrophy (FECD) and rescues
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