视网膜色素变性蛋白26抗体
规格：1mg/1ml
英文名： CERKL
别名： Ceramide kinase like protein; Ceramide kinase-like protein; CERKL; CERKL_HUMAN; Retinitis pigmentosa 26 (autosomal recessive); RP26.
分子量： 61kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CAMK1D
交叉反应：Human, Mouse, Rat, Sheep,
细胞定位：细胞核 细胞浆
视网膜色素变性蛋白26抗体产品介绍：background: This gene was initially identified as a locus (RP26) associated with an autosomal recessive form of retinitis pigmentosa (arRP) disease. This gene encodes a protein with ceramide kinase-like domains, however, the protein does not phosphorylate ceramide and its target substrate is currently unknown. This protein may be a negative regulator of apoptosis in photoreceptor cells. Mutations in this gene cause a form of retinitis pigmentosa characterized by autosomal recessive cone and rod dystrophy (arCRD). Alternative splicing of this gene results in multiple transcript variants encoding different isoforms and non-coding transcripts.[provided by RefSeq, May 2010]. Function: Has no detectable ceramide-kinase activity. Overexpression of CERKL protects cells from apoptosis in oxidative stress conditions. Subcellular Location: Cytoplasm. Nucleus, nucleolus. Note=Enriched in nucleoli. May shuttle between nucleus and cytoplasm. Isoform 5 is not enriched in the nucleoli. Isoform 2: Cytoplasm. Nucleus, nucleolus. Golgi apparatus, trans-Golgi network. Endoplasmic reticulum. Tissue Specificity: Isoform 1 and isoform 2 are expressed in ***** retina, liver and pancreas as well as in fetal brain, lung and kidney. Isoform 3 is expressed in视网膜色素变性蛋白26抗体 ***** retina as well as in fetal lung and liver. Isoform 4 is expressed in ***** retina, lung and kidney as well as in fetal lung and liver. Moderately expressed in retina, kidney, lung, testis, trachea, and pancreas. Weakly expressed in brain, placenta and liver. Post-translational modifications: Phosphorylated on serine residues. DISEASE: Defects in CERKL are the cause of retinitis pigmentosa type 26 (RP26) [MIM:608380]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their 视网膜色素变性蛋白26抗体condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP26 inheritance is autosomal recessive. Similarity: Contains 1 DAGKc domain. Gene ID: 375298 Database links: Entrez Gene: 375298 Human Omim: 608381 Human SwissProt: Q49MI3 Human Unigene: 715753 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
视网膜色素变性蛋白26抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.
研究领域：细胞生物 神经生物学 激酶和磷酸酶
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid