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Anti-PORCN, clone 15G12.1 (mouse monoclonal)

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货号：MABS21

品牌：Millipore 密理博

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重要规格表

Species Reactivity Key Applications Host Format Antibody Type
H, M, R WB, ICC M Purified Monoclonal Antibody
Description
Catalogue Number MABS21
Description Anti-PORCN Antibody, clone 15G12.1
Alternate Names
  • Probable protein-cysteine N-palmitoyltransferase porcupine
  • Protein MG61
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.
References
Product Information
Format Purified
Control
  • Mouse brain tissue lysate
Presentation Purified mouse monoclonal IgMκ in buffer containing PBS with 0.05% sodium azide.
Applications
Application Anti-PORCN Antibody, clone 15G12.1 is an antibody against PORCN for use in WB & IC.
Key Applications
  • Western Blotting
  • Immunocytochemistry
Application Notes Immunocytochemistry Analysis: A 1:500 dilution from a representative lot detected PORCN in HeLa and A431 cells.
Biological Information
Immunogen Linear peptide corresponding to human PORCN.
Clone 15G12.1
Concentration Please refer to the Certificate of Analysis for the lot-specific concentration.
Host Mouse
Isotype IgMκ
Species Reactivity
  • Human
  • Mouse
  • Rat
Antibody Type Monoclonal Antibody
Entrez Gene Number
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].
Gene Symbol
  • PORCN
  • MG61
  • PORC
  • PPN
Purification Method Purified by Ion-Exchange Chromatography
UniProt Number
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.
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).
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
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.
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.
Storage and Shipping Information
Storage Conditions Stable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size 100 µL
Transport Information
Supplemental Information
Specifications

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