货号：MABS21

品牌：Merck Millipore

规格：100UL

目录价：￥4438.00

市场价格：￥3772.30

会员价格：￥3550.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-PORCN Antibody, clone 15G12.1 | MABS21 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~62 kDa observed. Calculated molecular weight of 52 kDa. The actual MW should be observed at ~45-65 kDa. Helical membrane protein migration on SDS-PAGE may not exactly correlate with the calculated molecular weights (PNAS, 2009, 106:1760-1765). View All» Immunogen: Linear peptide corresponding to human PORCN. View All» Clone: 15G12.1 View All» Isotype: IgMκ View All» Background Information: 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. View All» Species Reactivity:

• Human

• Mouse

• Rat
  View All» Species Reactivity Note: Demonstrated to react with Human, Mouse, and Rat. View All» Application Notes: Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected PORCN in HeLa and A431 cells. View All» Control: Mouse brain tissue lysate View All» Quality Assurance: 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. View All» Purification Method: Purified by Ion Exchange Chromatography View All» Presentation: Purified mouse monoclonal IgMκ in buffer containing PBS with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q9H237 View All» Entrez Gene Number: NP_073736 View All» Gene Symbol:
  • PORCN

  • MG61

  • PORC

  • PPN
    View All» Alternate Names:
    • Probable protein-cysteine N-palmitoyltransferase porcupine

    • Protein MG61
      View All» Usage Statement: 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. View All» Key Applications:
      • Western Blotting

      • Immunocytochemistry
        View All» Entrez Gene Summary: 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]. View All» UniProt Summary: 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. View All» Product Name: Anti-PORCN, clone 15G12.1 View All» Concentration: 0.5 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Purified View All» Host: Mouse View All»
        © 金山科研平台是专业的授权总代理区域代理经销平台。
        © 如需询价，请加客服QQ：1749072012 、客服微信：jinshanbio，或发送邮件到1749072012@qq.com
        © 平台为生命科学研究相关领域提供一站式耗材试剂仪器解决方案和采购服务，数据资源基于CC协议。
        © 本文地址：https://16ao.com/thread-133183.htm
        © Merck Millipore代理MABS21 Anti-PORCN Antibody, clone 15G12.1;store at +2℃ to +8℃，产品报价联系微信jinshanbio