FITC标记的先天性红细胞生成异常性贫血蛋白1抗体

¥2980
LMAI Bio
中国/美国/欧洲
2022-03-24 10:31

上海联迈生物工程有限公司

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产品属性
供应商上海联迈生物工程有限公司
数量大量
靶点详见说明书
级别1
目录编号LM-7994R-FITC
克隆性多克隆
抗原来源Rabbit
保质期1年
抗体英文名Anti-CDAN1/FITC
抗体名Anti-CDAN1/FITC
标记物FITC标记
宿主Human, Mouse,
适应物种Human, Mouse,
免疫原详见说明书
亚型IGg
形态粉末、液体、冻干粉
应用范围IF=1:50-200
浓度1mg/ml
保存条件-20 °C
规格100ul
产品说明
FITC标记的先天性红细胞生成异常性贫血蛋白1抗体
英文名称Anti-CDAN1/FITC
中文名称FITC标记的先天性红细胞生成异常性贫血蛋白1抗体
别 名Alternative namesCDA1; CDAI; CDAN1; CDAN1_HUMAN; Codanin; Codanin-1; Codanin1; PRO1295.
规格价格100ul/2980元 购买 大包装/询价
说 明 书100ul
研究领域心血管 细胞生物 免疫学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse,
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56/130kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human CDAN1
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:
This gene encodes a protein that appears to play a role in nuclear envelope integrity, possibly related to microtubule attachments. Mutations in this gene cause congenital dyserythropoietic anemia type I, a disease resulting in morphological and functional abnormalities of erythropoiesis.

Function:
Might be involved in nuclear membrane integrity.

Subcellular Location:
Membrane; Multi-pass membrane protein.

Tissue Specificity:
Ubiquitously expressed. Isoform 3 is not found in erythroid cells.

DISEASE:
Defects in CDAN1 are the cause of congenital dyserythropoietic anemia type 1 (CDA1) [MIM:224120]. An autosomal recessive blood disorder characterized by morphological abnormalities of erythroblasts, ineffective erythropoiesis, macrocytic anemia and secondary hemochromatosis. It is occasionally associated with bone abnormalities, especially of the hands and feet (acrodysostosis), nail hypoplasia, and scoliosis. Ultrastructural features include internuclear chromatin bridges connecting some nearly completely separated erythroblasts and an abnormal appearance (spongy or Swiss-cheese appearance) of the heterochromatin in a high proportion of the erythroblasts.

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