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品种反应性 主要应用 宿主 格式 抗体类型 M, H WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 06-1106 Replaces 04-971 描述 Anti-Sonic Hedgehog Antibody Alternate Names - sonic hedgehog homolog (Drosophila)
- sonic hedgehog
- 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 Affinity Purified 控制 - Human fetal skeletal muscle tissue lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide. 应用 应用 Anti-Sonic Hedgehog Antibody detects level of Sonic Hedgehog & has been published & validated for use in WB, IH. 主要应用 - Western Blotting
应用说明 Western Blot (SNAP ID) Analysis: 1 µg/mL from a previous lot detected Sonic Hedgehog on 10 µg of human fetal skeletal muscle tissue lysate.
Immunohistochemistry Analysis: 1:500 dilution from a previous lot detected Sonic Hedgehog in colorectal carcinoma tissue.生物信息 免疫原品种 GST-tagged recombinant protein corresponding to the N-terminus of Sonic HedgeHog. 表位 N-terminus 宿主 Rabbit 特异性 This antibody recognizes the N-terminus of Sonic Hedgehog. 品种反应性 MouseHuman Species Reactivity Note Demonstrated to react with human and mouse.
抗体类型 Polyclonal Antibody Entrez基因编号 - NP_000184
Entrez基因汇总 This gene encodes a protein that is instrumental in patterning the early embryo. It has been implicated as the key inductive signal in patterning of the ventral neural tube, the anterior-posterior limb axis, and the ventral somites. Of three human proteins showing sequence and functional similarity to the sonic hedgehog protein of Drosophila, this protein is the most similar. The protein is made as a precursor that is autocatalytically cleaved; the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the developing embryo. Defects in this protein or in its signalling pathway are a cause of holoprosencephaly (HPE), a disorder in which the developing forebrain fails to correctly separate into right and left hemispheres. HPE is manifested by facial deformities. It is also thought that mutations in this gene or in its signalling pathway may be responsible for VACTERL syndrome, which is characterized by vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial and renal dysplasia, cardiac anomalies, and limb abnormalities. Additionally, mutations in a long range enhancer located approximately 1 megabase upstream of this gene disrupt limb patterning and can result in preaxial polydactyly. [provided by RefSeq]. 基因符号 - TPTPS
- SHH
- HHG-1
- HHG1
- HLP3
- HPE3
- MCOPCB5
- SMMCI
- TPT
纯化方法 Affinity Purfied 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 space by 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 Blot in human fetal skeletal muscle tissue lysate.
Western Blot Analysis: 0.1 µg/mL of this antibody detected Sonic Hedgehog on 10 µg of human fetal skeletal muscle 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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