Human SHH (C24II) Recombinant Protein (RPPB0903)

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주문
SKU:
RPPB0903
Product type:
Recombinant Protein
Size:
10ug
Species:
Human
Target:
SHH (C24II)
Synonyms:
Sonic Hedgehog
Synonyms:
MCOPCB5
Synonyms:
SMMCI
Synonyms:
HLP3
Source:
Escherichia Coli
Uniprot:
Q15465
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Description

Product Name:Human SHH (C24II) Recombinant Protein
Product Code:RPPB0903
Size:10µg
Species:Human
Target:SHH (C24II)
Synonyms:Sonic Hedgehog, MCOPCB5, SMMCI, HLP3, HPE3, Sonic Hedgehog (Drosophila) Homolog, Sonic Hedgehog Homolog (Drosophila), Sonic Hedgehog Homolog, Sonic Hedgehog Protein, TPTPS, HHG-1, HHG1, TPT, SHH (C24II).
Source:Escherichia Coli
Physical Appearance:Sterile filtered colorless solution.
Formulation:The SHH protein solution (1mg/ml) containing Phosphate buffered saline (pH7.4).
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 95% as determined by SDS-PAGE.
Amino Acid Sequence:MIIGPGRGFG KRRHPKKLTP LAYKQFIPNV AEKTLGASGR YEGKISRNSE RFKELTPNYN PDIIFKDEEN TGADRLMTQR CKDKLNALAI SVMNQWPGVK LRVTEGWDED GHHSEESLHY EGRAVDITTS DRDRSKYGML ARLAVEAGFD WVYYESKAHI HCSVKAENSV AAKSGG

Sonic Hedgehog is part of a small group of secreted proteins which are vital for development in both vertebrates and invertebrates. 3 mammalian hedgehog genes (sonic, desert, Indian) share about 60% homology. The Human Sonic Hedgehog is 99% homologous to the mouse gene. Sonic HedgeHog is a protein that is vital in guding the early embryo. It has been associated as the major inductive signal in patterning of the ventral neural tube, the anterior-posterior limb axis, and the ventral somites. Sonic HedgeHog binds to the patched receptor, which functions in association with smoothened, to activate the transcription of target genes. In the absence of sonic HedgeHog, patched receptor represses the constitutive signaling activity of smoothened. Sonic HedgeHog also regulates another factor, the gli oncogene. Sonic HedgeHog intercellular signal is essential for a various 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. Sonic HedgeHog exhibits both floor plate- and motor neuron-inducing activity. Mutations in a long-range Sonic HedgeHog enhancer located in an intron of the limb region 1 gene result in preaxial polydactyly.

SHH Human Recombinant produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 176 amino acids (24-197 a.a.) and having a molecular mass of 19.8kDa (The Cys at position 24 has been replaced with 2 Ile).The SHH is purified by proprietary chromatographic techniques.

UniProt Protein Function:SHH: 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. Interacts with HHATL/GUP1 which negatively regulates HHAT-mediated palmitoylation of the SHH N-terminus. N-product is active as a multimer. Expressed in fetal intestine, liver, lung, and kidney. Not expressed in adult tissues. Belongs to the hedgehog family.
UniProt Protein Details:

Protein type:Motility/polarity/chemotaxis; Cell cycle regulation; Oncoprotein; Cell development/differentiation

Chromosomal Location of Human Ortholog: 7q36

Cellular Component: extracellular matrix; extracellular space; cell surface; endoplasmic reticulum lumen; plasma membrane; extracellular region; nucleus; cytosol; lipid raft

Molecular Function:laminin-1 binding; peptidase activity; morphogen activity; protein binding; glycosaminoglycan binding; zinc ion binding; patched binding; calcium ion binding; glycoprotein binding

Biological Process: prostate gland development; central nervous system development; positive regulation of transcription, DNA-dependent; embryonic skeletal development; telencephalon regionalization; embryonic foregut morphogenesis; male genitalia development; neural crest cell migration; inner ear development; embryonic limb morphogenesis; hindbrain development; positive regulation of neuroblast proliferation; camera-type eye development; neuron fate commitment; myotube differentiation; intermediate filament organization; osteoblast development; positive regulation of skeletal muscle cell proliferation; positive regulation of cell division; regulation of proteolysis; positive regulation of transcription from RNA polymerase II promoter; embryonic digit morphogenesis; negative regulation of apoptosis; embryonic forelimb morphogenesis; axon guidance; granule cell precursor proliferation; ventral midline development; spinal cord dorsal/ventral patterning; thalamus development; positive regulation of striated muscle cell differentiation; negative regulation of transcription from RNA polymerase II promoter; palate development; Bergmann glial cell differentiation; negative regulation of T cell proliferation; positive regulation of cell proliferation; pancreas development; forebrain development; thyroid gland development; heart looping; vasculogenesis; negative regulation of cell migration; positive thymic T cell selection; intein-mediated protein splicing; regulation of odontogenesis; androgen metabolic process; spinal cord motor neuron differentiation; pattern specification process; regulation of cell proliferation; odontogenesis of dentine-containing teeth; negative regulation of cell differentiation; stem cell development; embryonic development; dorsal/ventral pattern formation; positive regulation of protein import into nucleus; ureteric bud branching; hindgut morphogenesis; lung development; negative regulation of alpha-beta T cell differentiation; heart development; T cell differentiation in the thymus; CD4-positive or CD8-positive, alpha-beta T cell lineage commitment; lymphoid progenitor cell differentiation; Wnt receptor signaling pathway through beta-catenin; embryonic pattern specification; proteolysis; positive regulation of T cell differentiation in the thymus; cell-cell signaling; embryonic digestive tract morphogenesis; midbrain development; ectoderm development; positive regulation of smoothened signaling pathway; positive regulation of oligodendrocyte differentiation; oligodendrocyte development; activation of hh target transcription factor; striated muscle development; endocytosis; positive regulation of skeletal muscle development; negative thymic T cell selection; patterning of blood vessels; branching morphogenesis of a tube; polarity specification of anterior/posterior axis; metanephros development; cell fate specification; embryonic hindlimb morphogenesis; positive regulation of Wnt receptor signaling pathway; dorsoventral neural tube patterning; smoothened signaling pathway; organ formation; hair follicle morphogenesis; thymus development; smoothened signaling pathway in regulation of granule cell precursor cell proliferation; positive regulation of immature T cell proliferation in the thymus; negative regulation of proteasomal ubiquitin-dependent protein catabolic process; formation of anatomical boundary; myoblast differentiation; limb bud formation; establishment of cell polarity; neuroblast proliferation; blood coagulation; cell development; positive regulation of alpha-beta T cell differentiation

Disease: Solitary Median Maxillary Central Incisor; Schizencephaly; Microphthalmia, Isolated, With Coloboma 5; Holoprosencephaly 3

NCBI Summary: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, Jul 2008]
UniProt Code:Q15465
NCBI GenInfo Identifier:6094283
NCBI Gene ID:6469
NCBI Accession:Q15465.1
UniProt Secondary Accession:Q15465,Q75MC9, A4D247,
UniProt Related Accession:Q15465
Molecular Weight:49,607 Da
NCBI Full Name:Sonic hedgehog protein
NCBI Synonym Full Names:sonic hedgehog
NCBI Official Symbol:SHH  
NCBI Official Synonym Symbols:TPT; HHG1; HLP3; HPE3; SMMCI; TPTPS; MCOPCB5  
NCBI Protein Information:sonic hedgehog protein; sonic hedgehog homolog
UniProt Protein Name:Sonic hedgehog protein
UniProt Synonym Protein Names:HHG-1Cleaved into the following 2 chains:Sonic hedgehog protein N-product; Sonic hedgehog protein C-product
Protein Family:Sonic hedgehog protein
UniProt Gene Name:SHH  
UniProt Entry Name:SHH_HUMAN
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