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品种反应性 主要应用 宿主 格式 抗体类型 R, H, M ICC, WB M Purified Monoclonal Antibody 描述 产品目录编号 MABS21 描述 Anti-PORCN Antibody, clone 15G12.1 Alternate Names - Protein MG61
- Probable protein-cysteine N-palmitoyltransferase porcupine
背景信息 PORCN is a multi-pass endoplasmic reticulum protein involved in the processing of wingless and other Wnt proteins that facilitate their secretion from Wnt-producing cells. Studies have shown mutations in the genes that encode for PORCN cause a pleiotropic X-linked dominant disorder, focal dermal hypolasia (FDH, also known as Goltz syndrome). Skeletal abnormalities such as syndactyly, ectordactyly, and brachydactyly, and some cases of osteopathia striata have also been attibuted to PORCN abnormalities. 产品信息 格式 Purified 控制 - Mouse brain tissue lysate
演示 Purified mouse monoclonal IgMκ in buffer containing PBS with 0.05% sodium azide. 应用 应用 Anti-PORCN Antibody, clone 15G12.1 is an antibody against PORCN for use in WB & IC. 主要应用 - Immunocytochemistry
- Western Blotting
应用说明 Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected PORCN in HeLa and A431 cells. 生物信息 免疫原品种 Linear peptide corresponding to human PORCN. 克隆 15G12.1 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 同种型 IgMκ 品种反应性 RatHumanMouse Species Reactivity Note Demonstrated to react with Human, Mouse, and Rat. 抗体类型 Monoclonal Antibody Entrez基因编号 - NP_073736
Entrez基因汇总 This gene belongs to the evolutionarily conserved porcupine (Porc) gene family. Genes of the Porc family encode endoplasmic reticulum proteins with multiple transmembrane domains. Porcupine proteins are involved in the processing of Wnt (wingless and int homologue) proteins. Multiple alternatively spliced transcript variants encoding distinct isoforms have been observed. [provided by RefSeq, Jul 2008]. 基因符号 - PPN
- PORCN
- MG61
- PORC
纯化方法 Purified by Ion Exchange Chromatography UniProt编号 - Q9H237
UniProt汇总 FUNCTION: Modulates the processing of Wnt proteins. Probable protein-cysteine N-palmitoyltransferase that palmitoylates Wnt family members.
SUBUNIT STRUCTURE: Interacts with WNT1, WNT3, WNT3A, WNT4, WNT5A, WNT5B, WNT6, WNT7A and WNT7B (By similarity).
SUBCELLULAR LOCATION: Endoplasmic reticulum membrane; Multi-pass membrane protein (By similarity).
TISSUE SPECIFICITY: Isoform 1 is expressed in fetal brain, brain, amygdala, caudate nucleus, cerebellum, hippocampus, pituitary, thalamus, heart, skeletal muscle and testis. Isoform 4 is expressed in amygdala, corpus callosum, hippocampus, spinal cord, kidney, liver, lung, spleen, uterus, testis. Isoform 2 and isoform 3 are expressed in substantia negra, spinal cord, heart and lung.
INVOLVEMENT IN DISEASE: Defects in PORCN are the cause of focal dermal hypoplasia (FODH) [MIM:305600]; also known as Goltz Gorlin syndrome. A rare congenital ectomesodermal disorder characterized by a combination of skin defects, skeletal abnormalities, and ocular anomalies. Affected individuals have patchy dermal hypoplasia, often in a distribution pattern following the Blaschko lines, and areas of subcutaneous fat herniation or deposition of fat into the dermis. In addition, sparse and brittle hair, hypoplastic nails and papillomas have been described. Skeletal abnormalities usually comprise syndactyly, ectrodactyly, and brachydactyly, and in some cases osteopathia striata has been seen. Patients frequently have ocular anomalies, including microphthalmia/ anophthalmia, coloboma, pigmentary and vascularization defects of the retina. Dental abnormalities are often present.
SEQUENCE SIMILARITIES: Belongs to the membrane-bound acyltransferase family. Porcupine subfamily.
SEQUENCE CAUTION: The sequence AAA74510.1 differs from that shown. Reason: The sequence differs from that shown upstream of position 63 for unknown reasons.产品使用声明 质量保证 Evaluated by Western Blot in mouse brain tissue lysate.
Western Blot Analysis: A 1:1,000 dilution of this antibody detected PORCN in 10 µg of mouse brain tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µL
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