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Product Name Anti-IFT80/AP Chinese Name 碱性磷酸酶(AP)标记的细胞纤毛内转运同源蛋白80抗体 Alias ATD2; Ift80; IFT80_HUMAN; Intraflagellar transport 80 homolog (Chlamydomonas); Intraflagellar transport protein 80 homolog; KIAA1374; WD repeat domain 56; WD repeat-containing protein 56; WDR56. Research Area Cell biology immunology Transporter Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, ) Applications WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 88kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human IFT80 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage 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. Product Detail background:
IFT80 is a encoded by this gene is part of the intraflagellar transport complex B and is necessary for the function of motile and sensory cilia. Defects in this gene are a cause of asphyxiating thoracic dystrophy 2 (ATD2). Three transcript variants encoding two different isoforms have been found for this gene.
Function:
Component of the intraflagellar transport (IFT) complex B, which is essential for the development and maintenance of motile and sensory cilia.
Subcellular Location:
Cytoplasm. Cytoplasm, cytoskeleton, cilium basal body (By similarity). Cytoplasm, cytoskeleton, cilium axoneme (By similarity). Note=Basal body and ciliary axoneme (By similarity).
DISEASE:
Asphyxiating thoracic dystrophy 2 (ATD2) [MIM:611263]: An autosomal recessive chondrodysplasia characterized by a severely constricted thoracic cage, short-limbed short stature, and polydactyly. It often leads to death in infancy because of respiratory insufficiency. Retinal degeneration, cystic renal disease and hepatic disease can be present in affected individuals who survive early childhood. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Contains 7 WD repeats.
Database links:
UniProtKB/Swiss-Prot: Q9P2H3.3
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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