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Mouse Monoclonal Shh antibody [8G3] (STJ98379)
Supplier: St John’s Laboratory Ltd.
Recommended applications: WB, ELISA
Recommended dilution: WB 1:500-1:2000; ELISA 1:10000
Recommended protocols: check protocols
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Check alternative names for the antibodyExpand
SHH antibody,|HHG 1 antibody|HHG-1 antibody|HHG1 antibody|HLP 3 antibody|HLP3 antibody|Holoprosencephaly 3 antibody|HPE 3 antibody|HPE3 antibody|MCOPCB5 antibody|shh antibody|SHH_HUMAN antibody|SMMC I antibody|SMMCI antibody|Sonic Hedgehog (Drosophila) homolog antibody|sonic hedgehog homolog (Drosophila) antibody|Sonic hedgehog homolog antibody|Sonic hedgehog protein antibody|Sonic hedgehog protein C-product antibody|TPT antibody|TPTPS antibody|Anti-Sonic Hedgehog antibody [EP1190Y] (ab53281)
SCBT cat No: To be updated
Shh Monoclonal Antibody
|Catalogue No.|| |
Shh Monoclonal Antibody detects endogenous levels of Shh protein.
Purified recombinant fragment of human Shh expressed in E Coli
|Recommended dilution|| |
WB 1:500-1:2000; ELISA 1:10000
Shh Antibody was tube-contained. Ascitic fluid containing 0.03% sodium azide.
Shh Antibody was purified using affinity purification.
-20 Celsius degree. Avoid repeated freeze/thaw cycles.
|Alternative antibody names|| |
Sonic hedgehog protein antibody, SHH antibody, HHG-1 antibody
|Protein names|| |
Sonic hedgehog protein , SHH , HHG-1
|Protein function|| |
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. Activates the transcription of target genes by interacting with its receptor PTCH1 to prevent normal inhibition by PTCH1 on the constitutive signaling activity of SMO (By similarity).
|Protein tissue specificity|| |
Expressed in fetal intestine, liver, lung, and kidney. Not expressed in adult tissues.
|Involvement in disease|| |
Microphthalmia, isolated, with coloboma, 5 (MCOPCB5) [MIM:611638]: A 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, and other abnormalities 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). . Note: The disease is caused by mutations affecting the gene represented in this entry.; Holoprosencephaly 3 (HPE3) [MIM:142945]: A 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 holoprosencephaly type 3 cases are apparently sporadic, although clear examples of autosomal dominant inheritance have been described. . Note: The disease is caused by mutations affecting the gene represented in this entry.; Solitary median maxillary central incisor (SMMCI) [MIM:147250]: Rare dental anomaly characterized by the congenital absence of one maxillary central incisor. . Note: The disease is caused by mutations affecting the gene represented in this entry.; Triphalangeal thumb-polysyndactyly syndrome (TPTPS) [MIM:174500]: Autosomal dominant syndrome. It is characterized by a wide spectrum of pre- and post-axial abnormalities due to altered SHH expression pattern during limb development. . Note: The gene represented in this entry is involved in disease pathogenesis. Mutations located in intron 5 of LMBR1 disrupt a long-range, cis-regulatory element of SHH expression.; Preaxial polydactyly 2 (PPD2) [MIM:174500]: Polydactyly consists of duplication of the distal phalanx. The thumb in PPD2 is usually opposable and possesses a normal metacarpal. . Note: The gene represented in this entry is involved in disease pathogenesis. Mutations located in intron 5 of LMBR1 disrupt a long-range, cis-regulatory element of SHH and result in abnormal, ectopic SHH expression with pathological consequences (PubMed:12837695). .; Hypoplasia or aplasia of tibia with polydactyly (THYP) [MIM:188740]: An autosomal dominant disease characterized by hypoplastic or absent tibia, and polydactyly. . Note: The gene represented in this entry is involved in disease pathogenesis. Mutations located in intron 5 of LMBR1 disrupt a long-range, cis-regulatory element of SHH and result in abnormal, ectopic SHH expression with pathological consequences. .; Laurin-Sandrow syndrome (LSS) [MIM:135750]: A rare autosomal dominant disorder characterized by polysyndactyly of hands and/or feet, mirror image duplication of the feet, nasal defects, and loss of identity between fibula and tibia. Some patients do not have nasal abnormalities (segmental Laurin-Sandrow syndrome). . Note: The gene represented in this entry is involved in disease pathogenesis. Abnormal SHH limb expression with pathological consequences is caused by duplications (16-75 kb) involving the ZPA regulatory sequence (ZRS), a SHH long-range cis-regulatory element, located in LMBR1 intron 5 (PubMed:24456159).
|Protein sequence and domain|| |
The sonic hedgehog protein N-product binds calcium and zinc ions; this stabilizes the protein fold and is essential for protein-protein interactions mediated by this domain. / Belongs to the hedgehog family.
|Protein post-translational modifications|| |
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. / N-palmitoylation of Cys-24 by HHAT is required for N-product multimerization and full activity.
|Protein cellular localization|| |
Secreted > extracellular space / Cell membrane / Lipid-anchor
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St John’s Laboratory Ltd.
|Product type|| |
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