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DMPK Polyclonal Antibody

说明书

BYab-14731

  • 20uL ¥680 50uL ¥1350 100uL ¥1800
  • 货期: 3个工作日

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宿主
反应性
Human;Rat;Mouse;
应用
WB;ELISA
分子量(DA)
免疫原
The antiserum was produced against synthesized peptide derived from human DMPK. AA range:11-60
特异性
DMPK Polyclonal Antibody detects endogenous levels of DMPK protein.
来源
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
纯化工艺(Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
背景(Background)
The protein encoded by this gene is a serine-threonine kinase that is closely related to other kinases that interact with members of the Rho family of small GTPases. Substrates for this enzyme include myogenin, the beta-subunit of the L-type calcium channels, and phospholemman. The 3' untranslated region of this gene contains 5-38 copies of a CTG trinucleotide repeat. Expansion of this unstable motif to 50-5,000 copies causes myotonic dystrophy type I, which increases in severity with increasing repeat element copy number. Repeat expansion is associated with condensation of local chromatin structure that disrupts the expression of genes in this region. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Jul 2016],
功能
catalytic activity:ATP + a protein = ADP + a phosphoprotein.,cofactor:Magnesium.,disease:Defects in DMPK are the cause of myotonic dystrophy 1 (DM1) [MIM:160900]; also known as Steinert disease. DM is an autosomal dominant neurodegenerative disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness, and cardiac arrhythmias. DM patients show decreased levels of kinase expression inversely related to repeat length. The minimum estimated incidence is 1 in 8'000 live births. DM1 is caused by a CTG expansion in the 3'-UTR of the DMPK gene. The repeat length usually increases in successive generations, but not always.,enzyme regulation:Activated in response to G protein second messengers. Maintained in an inactive conformation by the negative autoregulatory C-terminal coiled-coil region. Coiled-coil mediate
基因名称(Gene Name)
蛋白名称
简称
其他名称
Fields
人基因ID
人蛋白质序列数据库
小鼠基因ID
小鼠蛋白质序列数据库
大鼠基因ID
大鼠蛋白质序列数据库
细胞定位
组织表达
储存(Storage)
-20°C/1 year

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DMPK Polyclonal Antibody

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