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Sonic Hedgehog (DGR14578) Rabbit mAb
db13299
Datasheet
- Background 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]
- Immunogen A synthetic peptide of human Sonic Hedgehog
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Gene ID
6469
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Swiss Prot
Q15465
- Synonyms TPT; HHG1; HLP3; HPE3; SMMCI; TPTPS; MCOPCB5
- Reactivity Human
- Application WB, IHC-P, ICC/IF, FC
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Recommended dilution
WB: 1:1000-1:5000
IHC-P: 1:200-1:2000
ICC/IF: 1:200-1:500
FC: 1:10-1:100 - Calculated MW 50 kDa
- Observed MW 50,27 kDa
- Host species Rabbit
- Clonality Monoclonal
- Clonality No. DGR14578
- Isotype IgG
- Purity Affinity Purification
- Conjugation Un-conjugated
- Storage/Stability Store at -20°C. Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.
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Western blot analysis of extracts from Human fetal kidney tissue using db13299 at 1:1000. -
Western blot analysis of extracts from A549 cells using db11476 at 1:4000.
订购信息
- Package Price (RMB)
- 10μL 398
- 20μL 698
- 50μL 1398
- 100μL 2398
- 货期：现货
相关产品
For research use only. Not intended for diagnostic.
