FITC标记的兜甲蛋白抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的兜甲蛋白抗体

FITC标记的兜甲蛋白抗体

商家询价

产品名称: FITC标记的兜甲蛋白抗体

英文名称: Anti-LOR/Loricrin/FITC

产品编号: HZ-18340R-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: ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-LOR/Loricrin/FITC Conjugated antibody

FITC标记的兜甲蛋白抗体

 

英文名称 Anti-LOR/Loricrin/FITC
中文名称 FITC标记的兜甲蛋白抗体
别    名 LOR; LOR protein; LORI_HUMAN; Loricrin; LRN; MGC111513; OTTHUMP00000015823.  
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  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Horse, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 26kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LOR/Loricrin
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 loricrin, a major protein component of the cornified cell envelope found in terminally differentiated epidermal cells. Mutations in this gene are associated with Vohwinkel's syndrome and progressive symmetric erythrokeratoderma, both inherited skin diseases. [provided by RefSeq, Jul 2008]

Function:
Major keratinocyte cell envelope protein.

Subcellular Location:
Cytoplasm. Nucleus;nucleoplasm.

Post-translational modifications:
Substrate of transglutaminases. Some glutamines and lysines are cross-linked to other loricrin molecules and to SPRRs proteins.
Contains inter- or intramolecular disulfide-bonds.

DISEASE:
Defects in LOR are a cause of progressive symmetric erythrokeratodermia (PSEK) [MIM:133200]. Erythrokeratodermas are a group of disorders characterized by widespread erythematous plaques, either stationary or migratory, associated with features that include palmoplantar keratoderma. PSEK is characterized by erythematous and hyperkeratotic plaques.
Defects in LOR are the cause of Vohwinkel syndrome with ichthyosis (VSI) [MIM:604117]; also known as loricrin keratoderma (LK) or mutilating keratoderma with ichthyosis. VSI is an ichthyotic variant of Vohwinkel syndrome (VS) characterized by progressive symmetric erythrokeratoderma or congenital ichthyosiform erythroderma born as a collodion baby. Common clinical features include hyperkeratosis of the palms and soles with digital constriction.

Database links:

Entrez Gene: 4014 Human

Entrez Gene: 16939 Mouse

Entrez Gene: 502541 Rat

Omim: 152445 Human

SwissProt: P23490 Human

SwissProt: P18165 Mouse

Unigene: 251680 Human

Unigene: 1121 Mouse



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

这个基因编码loricrin,在终末分化表皮细胞中发现的角化细胞膜上的主要蛋白成分。该基因的突变与Vohwinkel综合征和进行性对称性红角皮病有关,这两种疾病都是遗传性皮肤病。[ RefSeq,JUL 2008 ]