04-971 | Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit | 04-971

04-971
100 µL  
Retrieving price...
Price could not be retrieved
Minimum Quantity needs to be mulitiple of
Upon Order Completion More Information
You Saved ()
 
Request Pricing
Limited AvailabilityLimited Availability
In Stock 
Discontinued
Limited Quantities Available
Availability to be confirmed
    Remaining : Will advise
      Remaining : Will advise
      Will advise
      Contact Customer Service

      Special Offers

       

      Contact Customer Service

      Click To Print This Page

      Overview

      Replacement Information

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, IH(P), FC, IPRbPurifiedMonoclonal Antibody
      Description
      Catalogue Number04-971
      DescriptionAnti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit | 04-971
      Alternate Names
      • sonic hedgehog homolog (Drosophila)
      • sonic hedgehog (Drosophila) homolog
      • sonic hedgehog
      Background InformationSonic Hedgehog Protein (SHH, VHH-1) belongs to hedgehog protein family which includes Indian Hh, and Desert Hh. Hh family is involved in the cell fate and patterning during embryonic development, homeostasis, and adult tissue renewal. Similar to other member in the family, SHH binds to the patched (PTC) cell surface receptor, releasing the signal transducer Smoothened (Smo) to transmit the Hh signal into the cell and activate transcription of the target gene. Precursor SHH is autocatlytically cleaved into two subunits, N-terminal and C-terminal products. Soluble N-terminal product is involved in the signaling activity, while C-product displays an autoproteolysis activity on the precursor and a cholesterol transferase activity on the N-terminal product. C-terminal product attaches a cholesterol moiety to the N-terminal product, preventing N-terminal product diffusion within the developing embryo. A defect in SHH has been linked in holoprosencephaly type 3 (HPE3), in which developing forebrain fails to separate into right and left hemispheres, and ocular coloboma.
      References
      Product Information
      FormatPurified
      Control
      • Fetal liver membrane, human kidney tissue.
      PresentationRabbit monoclonal IgG in buffer containing 50 mM Tris-Glycine (pH7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
      Applications
      ApplicationPlease note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target.
      Key Applications
      • Western Blotting
      • Immunohistochemistry (Paraffin)
      • Flow Cytometry
      • Immunoprecipitation
      Application NotesFlow Cytometry: A 1:40 dilution of a previous lot was used in flow cytometry.

      Immunoprecipitation: A 1:70 dilution of a previous lot was used in immunoprecipitation.

      Immunohistochemistry (paraffin): A 1:100 dilution of a previous lot was used in immunohistochemsitry.
      Biological Information
      ImmunogenA synthetic peptide corresponding to residues within c-product subunit of human SHH protein was used as an immunogen.
      EpitopeC-product
      CloneEP1190Y
      HostRabbit
      SpecificityThis antibody is specific for both full length (50 kDa) and c-product subunit (27 kDa) of human SHH protein. Sonic hedgehog protein C-product is secreted and can be found in the extracellular space.
      IsotypeIgG
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • HHG-1
      • HHG1
      • HLP3
      • HPE3
      • MCOPCB5
      • SMMCI
      • TPT
      • TPTPS
      UniProt Number
      UniProt SummaryFUNCTION: Binds to the patched (PTC) receptor, which functions in association with smoothened (SMO), to activate the transcription of target genes. In the absence of SHH, PTC represses the constitutive signaling activity of SMO. Also regulates another target, the gli oncogene. Intercellular signal essential for a variety of patterning events during development: signal produced by the notochord that induces ventral cell fate in the neural tube and somites, and the polarizing signal for patterning of the anterior-posterior axis of the developing limb bud. Displays both floor plate- and motor neuron-inducing activity. The threshold concentration of N-product required for motor neuron induction is 5-fold lower than that required for floor plate induction By similarity.

      SUBUNIT STRUCTURE: Interacts with HHATL/GUP1 which negatively regulates HHAT-mediated palmitoylation of the SHH N-terminus. N-product is active as a multimer By similarity.

      SUBCELLULAR LOCATION: Sonic hedgehog protein C-product: Secreted › extracellular spaceBy similarity. Note= The C-terminal peptide diffuses from the cell By similarity.

      Sonic hedgehog protein N-product: Cell membrane; Lipid-anchorBy similarity. Note= The N-product either remains associated with lipid rafts at the cell surface, or forms freely diffusible active multimers with its hydrophobic lipid-modified N- and C-termini buried inside By similarity.

      TISSUE SPECIFICITY: Expressed in fetal intestine, liver, lung, and kidney. Not expressed in adult tissues.

      PTM: The C-terminal domain displays an autoproteolysis activity and a cholesterol transferase activity. Both activities result in the cleavage of the full-length protein and covalent attachment of a cholesterol moiety to the C-terminal of the newly generated N-terminal fragment (N-product). The N-product is the active species in both local and long-range signaling, whereas the C-product has no signaling activity.

      Cholesterylation is required for N-product targeting to lipid rafts and multimerization By similarity.

      N-palmitoylation of Cys-24 by HHAT is required for N-product multimerization and full activity By similarity.

      INVOLVEMENT IN DISEASE: Defects in SHH are the cause of microphthalmia isolated with coloboma type 5 (MCOPCB5) [MIM:611638]. Microphthalmia is a clinically heterogeneous disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues. Ocular abnormalities like opacities of the cornea and lens, scaring of the retina and choroid, cataract and other abnormalities like cataract may also be present. Ocular colobomas are a set of malformations resulting from abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure).

      Defects in SHH are the cause of holoprosencephaly type 3 (HPE3) [MIM:142945]. Holoprosencephaly (HPE) [MIM:236100] is the most common structural anomaly of the brain, in which the developing forebrain fails to correctly separate into right and left hemispheres. Holoprosencephaly is genetically heterogeneous and associated with several distinct facies and phenotypic variability. The majority of HPE3 cases are apparently sporadic, although clear exemples of autosomal dominant inheritance have been described. Interestingly, up to 30% of obligate carriers of HPE3 gene in autosomal dominant pedigrees are clinically unaffected.

      Defects in SHH are a cause of solitary median maxillary central incisor (SMMCI) [MIM:147250]. SMMCI is a rare dental anomaly characterized by the congenital absence of one maxillary central incisor.

      Defects in SHH are the cause of triphalangeal thumb-polysyndactyly syndrome (TPTPS) [MIM:174500]. TPTPS is an autosomal dominant syndrome characterized by a wide spectrum of pre- and post-axial abnormalities due to altered SHH expression pattern during limb development. TPTPS mutations have been mapped to the 7q36 locus in the LMBR1 gene which contains in its intron 5 a long-range cis-regulatory element of SHH expression.

      SEQUENCE SIMILARITIES: Belongs to the hedgehog family.

      MASS SPECTROMETRY: Molecular weight is 19.560 Da from positions 24 - 197. Determined by ESI. Soluble N-product, purified from insect cells.

      Molecular weight is 20.167 Da from positions 24 - 197. Determined by ESI. Membrane-bound N-product, purified from insect cells.

      Molecular Weight50 kDa & 27 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting on fetal liver membrane lysate.

      Western Blotting Analysis: 1:2,000 dilution of this antibody detected full length and c-product subunit of sonic hedgehog in fetal liver membrane 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 ConditionsStable for 1 year at -20ºC from date of receipt.
      Handling Recommendations: Upon receipt, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance. Note: Variability in freezer temperatures below -20°C may cause glycerolcontaining solutions to become frozen during storage.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Sonic Hedgehog (C-product), clone EP1190Y - 2459509 2459509
      Anti-Sonic Hedgehog (C-product), clone EP1190Y, Rabbit Monoclonal - 2148275 2148275
      Anti-Sonic Hedgehog (C-product), clone EP1190Y, Rabbit Monoclonal - NG1614431 NG1614431
      Anti-Sonic Hedgehog (C-product), clone EP1190Y, Rabbit Monoclonal - NG1754806 NG1754806
      Anti-Sonic Hedgehog (C-product), clone EP1190Y, Rabbit Monoclonal - NG1853770 NG1853770
      Anti-Sonic Hedgehog (C-product), clone EP1190Y, Rabbit Monoclonal - NG1940548 NG1940548
      Anti-Sonic Hedgehog (C-product), clone EP1190Y, Rabbit Monoclonal -2607891 2607891

      Related Products & Applications

      Related Products

      Catalogue Number Description  
      06-1106 Anti-Sonic Hedgehog Antibody Show Pricing & Availability

      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies