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先天性紅細(xì)胞生成異常性貧血蛋白1抗體
  • 產(chǎn)品貨號(hào):
    BN41294R
  • 中文名稱:
    先天性紅細(xì)胞生成異常性貧血蛋白1抗體
  • 英文名稱:
    Rabbit anti-CDAN1 Polyclonal antibody
  • 品牌:
    Biorigin
  • 貨號(hào)

    產(chǎn)品規(guī)格

    售價(jià)

    備注

  • BN41294R-100ul

    100ul

    ¥2360.00

    交叉反應(yīng):Mouse(predicted:Human) 推薦應(yīng)用:WB,IHC-P,IHC-F,IF,ELISA

  • BN41294R-200ul

    200ul

    ¥3490.00

    交叉反應(yīng):Mouse(predicted:Human) 推薦應(yīng)用:WB,IHC-P,IHC-F,IF,ELISA

產(chǎn)品描述

英文名稱CDAN1
中文名稱先天性紅細(xì)胞生成異常性貧血蛋白1抗體
別    名Alternative namesCDA1; CDAI; CDAN1; CDAN1_HUMAN; Codanin; Codanin-1; Codanin1; PRO1295.  
研究領(lǐng)域心血管  細(xì)胞生物  免疫學(xué)  
抗體來(lái)源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Mouse,  (predicted: Human, )
產(chǎn)品應(yīng)用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復(fù))
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量56/130kDa
細(xì)胞定位細(xì)胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human CDAN1:1175-1227/1227 
亞    型IgG
純化方法affinity purified by Protein A
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹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.

SWISS:
Q8IWY9

Gene ID:
146059

Database links:

Entrez Gene: 146059 Human

Entrez Gene: 68968 Mouse

Entrez Gene: 311348 Rat

Omim: 607465 Human

SwissProt: Q8IWY9 Human

SwissProt: Q8CC12 Mouse

Unigene: 599232 Human

Unigene: 2289 Mouse



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














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