- 产品信息
商品描述
靶点-Target
DMD
反应性
Human,Mouse,Rat
应用
IHC,IF
宿主物种-Host Species
Rabbit
同种型-Isotype
IgG
修饰-Modification
Unmodified
MW(Observed)
405kD
推荐稀释比
IHC 1:50-300;IF 1:50-200
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
纯化工艺
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
浓度
1 mg/ml
克隆性
Polyclonal
免疫原
Synthesized peptide derived from part region of human protein
特异性
DMD Polyclonal Antibody detects endogenous levels of protein.
基因名称
DMD
蛋白名称
Dystrophin
Organism-1
Human
基因ID-1
1756
SwissProt-1
P11532
Organism-2
Mouse
SwissProt-2
P11531
Organism-3
Rat
SwissProt-3
P11530
背景
dystrophin(DMD) Homo sapiens The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as enc
细胞定位
Cell membrane, sarcolemma ; Peripheral membrane protein ; Cytoplasmic side . Cytoplasm, cytoskeleton . Cell junction, synapse, postsynaptic cell membrane . In muscle cells, sarcolemma localization requires the presence of ANK2, while localization to costameres requires the presence of ANK3. Localizes to neuromuscular junctions (NMJs). In adult muscle, NMJ localization depends upon ANK2 presence, but not in newborn animals. .





