FITC标记的SCYL结合蛋白1抗体-抗体-抗体-生物在线
FITC标记的SCYL结合蛋白1抗体

FITC标记的SCYL结合蛋白1抗体

商家询价

产品名称： FITC标记的SCYL结合蛋白1抗体

英文名称： Anti-SCYL1BP1/FITC

产品编号： HZ-6643R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： IF=1:50-200

上海沪震实业有限公司
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 Rabbit Anti-SCYL1BP1/FITC Conjugated antibody

FITC标记的SCYL结合蛋白1抗体

 

英文名称 Anti-SCYL1BP1/FITC
中文名称 FITC标记的SCYL结合蛋白1抗体
别    名 hNTKL BP1; N terminal kinase like binding protein 1; NTKL binding protein 1; NTKL BP1; NTKLBP 1; NTKLBP1; SCY1 like 1 binding protein 1; SCYL1 binding protein 1; SCYL1 BP1; GORAB_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 45kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCYL1BP1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
Defects in GORAB are the cause of geroderma osteodysplasticum (GO) [MIM:231070]; also known as gerodermia osteodysplastica or Walt Disney dwarfism. GO is a rare autosomal recessive disorder characterized by lax, wrinkled skin, joint laxity and a typical face with a prematurely aged appearance. Skeletal signs include severe osteoporosis leading to frequent fractures, malar and mandibular hypoplasia and a variable degree of growth retardation.

Subunit:
Interacts with SCYL1 (By similarity). Interacts with RCHY1 and RAB6A/RAB6.

Subcellular Location:
Cytoplasm. Golgi apparatus.

DISEASE:
Defects in GORAB are the cause of geroderma osteodysplasticum (GO) [MIM:231070]; also known as gerodermia osteodysplastica or Walt Disney dwarfism. GO is a rare autosomal recessive disorder characterized by lax, wrinkled skin, joint laxity and a typical face with a prematurely aged appearance. Skeletal signs include severe osteoporosis leading to frequent fractures, malar and mandibular hypoplasia and a variable degree of growth retardation.

Similarity:
Belongs to the GORAB family.

Database links:

UniProtKB/Swiss-Prot: Q5T7V8.1



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
   

GORAB的缺陷是皮肤硬皮病（GO）的原因[MIM：231070 ]；也称为骨性皮肤病或沃尔特迪士尼侏儒症。GO是一种罕见的常染色体隐性遗传疾病，其特征是松弛、皱褶皮肤、关节松弛和典型的面部衰老。骨标志包括严重的骨质疏松症，导致频繁骨折，颧骨和下颌骨发育不全和不同程度的生长迟缓。