英文名称
Cytokeratin 13 Recombinant Rabbit mAb
英文别名
CK13; K13; WSN2; Krt-1.13; Krt1-13; K1C13_HUMAN; KRT13; Cytokeratin-13 (CK-13); Keratin-13 (K13); K1C13_MOUSE; 47 kDa cytokeratin; keratin 13; keratin 13, type I; keratin, type I cytoskeletal 13; cytokeratin 13
免疫原
A synthesized peptide derived from human Cytokeratin 13: 121-155/458
纯化方法
affinity purified by Protein A
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
The protein encoded by this gene is a member of the keratin gene family. The keratins are intermediate filament proteins responsible for the structural integrity of epithelial cells and are subdivided into cytokeratins and hair keratins. Most of the type I cytokeratins consist of acidic proteins which are arranged in pairs of heterotypic keratin chains. This type I cytokeratin is paired with keratin 4 and expressed in the suprabasal layers of non-cornified stratified epithelia. Mutations in this gene and keratin 4 have been associated with the autosomal dominant disorder White Sponge Nevus. The type I cytokeratins are clustered in a region of chromosome 17q21.2. Alternative splicing of this gene results in multiple transcript variants; however, not all variants have been described. [provided by RefSeq, Jul 2008].
蛋白名
Keratin, type I cytoskeletal 13
亚基
Heterotetramer of two type I and two type II keratins. keratin-13 is generally associated with keratin-4.
组织特异性
Defects in KRT13 are a cause of white sponge nevus of cannon (WSN) . WSN is a rare autosomal dominant disorder which predominantly affects non-cornified stratified squamous epithelia. Clinically, it is characterized by the presence of soft, white, and spongy plaques in the oral mucosa. The characteristic histopathologic features are epithelial thickening, parakeratosis, and vacuolization of the suprabasal layer of oral epithelial keratinocytes. Less frequently the mucous membranes of the nose, esophagus, genitalia and rectum are involved.
疾病
White sponge nevus of cannon (WSN) [MIM:193900]: Rare autosomal dominant disorder which predominantly affects non-cornified stratified squamous epithelia. Clinically, it is characterized by the presence of soft, white, and spongy plaques in the oral mucosa. The characteristic histopathologic features are epithelial thickening, parakeratosis, and vacuolization of the suprabasal layer of oral epithelial keratinocytes. Less frequently the mucous membranes of the nose, esophagus, genitalia and rectum are involved. Note=The disease is caused by mutations affecting the gene represented in this entry.
相似性
Belongs to the intermediate filament family.