merck millipore,默克密理博,04-971,Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit,04-971

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merck millipore,默克密理博,04-971,Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit,04-971
产品名称：Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit, 04-971
产品型号：04-971
Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target.

merck millipore,默克密理博,04-971,Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit,04-971

产品介绍

merck millipore,默克密理博,04-971,Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit,04-971

重要规格表

品种反应性	主要应用	宿主	格式	抗体类型
H	FC, WB	Rb	Purified	Monoclonal Antibody

描述
产品目录编号	04-971
描述	Anti-Sonic Hedgehog (C-product) Antibody, clone EP1190Y, Rabbit \| 04-971
Alternate Names	sonic hedgehog sonic hedgehog homolog (Drosophila) sonic hedgehog (Drosophila) homolog
背景信息	Sonic 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.

产品信息
格式	Purified
控制	Fetal liver membrane, human kidney tissue.
演示	Rabbit 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.

应用
应用	Please note that this product will not be available for sale after March 15, 2015. Please select one of the other antibodies against this target.
主要应用	Flow Cytometry Western Blotting
应用说明	Flow 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.

生物信息
免疫原品种	A synthetic peptide corresponding to residues within c-product subunit of human SHH protein was used as an immunogen.
表位	C-product
克隆	EP1190Y
宿主	Rabbit
特异性	This 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.
同种型	IgG
品种反应性	Human
Species Reactivity Note	Human
抗体类型	Monoclonal Antibody
Entrez基因编号	NP_990152.1
基因符号	TPTPS HHG-1 HHG1 HLP3 HPE3 MCOPCB5 SMMCI TPT
UniProt编号	Q15465
UniProt汇总	FUNCTION: 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.

产品使用声明
质量保证	Evaluated 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.
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 -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.

储存和货运信息

存储条件

Stable 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.