产品货号 : mlR18340
英文名称 : LOR/Loricrin
中文名称 : 兜甲蛋白抗体
别 名 : LOR; LOR protein; LORI_HUMAN; Loricrin; LRN; MGC111513; OTTHUMP00000015823.
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 : WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 : 26kDa
细胞定位 : 细胞核 细胞浆
性 状 : Lyophilized or Liquid
浓 度 : 1mg/ml
免 疫 原 : KLH conjugated synthetic peptide derived from human LOR/Loricrin:251-312/312
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.
PubMed : PubMed
产品介绍 : 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.
SWISS:
P23490
Gene ID:
4014
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.