Human SHH/HHG1/ HLP3 ORF/cDNA clone-Adeno-associate virus(AAV) particle (NM_000193.3)

Product information

Catalog No.	Product Name	AAV serotype	AAV Grade	AAV quantity
vGMAAV000001	Human SHH Adeno-associate virus(AAV) particle	AAV1, AAV2, AAV2 variant (Y444F), AAV2 variant (Y272F, Y444F, Y500F, Y730F), AAV2 variant (Y444F, Y730F, Y500F, Y272F, Y704F, Y252F), AAV2 variant(AAV2.7m8), AAV5, AAV6, AAV8, AAV8-1m, AAV8-2m, AAV8 variant (Y733F, Y447F, Y275), AAV9, AAV-Rh.10, AAV-DJ, AAV-DJ/8, AAV-Retro (Retrograde), AAV9-PHP.B, AAV9-PHP.eB, AAV9-PHP.S, AAV-BR1, AAV-2i8, AAV-SIG, AAV-VEC, AAV4, AAV6.2, AAV6.2FF	Pilot Grade	1.0E+12VG/ml
				5.0E+12VG/ml
				1E+13VG/ml
				5E+13VG/ml
				1E+14VG/ml
			Research Grade	1.0E+12VG/ml
				5.0E+12VG/ml
				1E+13VG/ml
				5E+13VG/ml
				1E+14VG/ml
			GMP-like Grade	inquiry
			GMP Grade	inquiry

Product Description

Catalog ID	vGMAAV000001
Gene Name	SHH
Accession Number	NM_000193.3
Gene ID	6469
Species	Human
Product Type	Adeno-associate virus(AAV) particle (overexpression)
Insert Length	1389 bp
Gene Alias	HHG1, HLP3, HPE3, MCOPCB5, ShhNC, SMMCI, TPT, TPTPS
Fluorescent Reporter	EGFP
Mammalian Cell Selection
Fusion Tag	Null
Promoter	CMV
Resistance	Amplicin

Reference

Data / case study

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Associated products

Category	Cat No.	Products Name
Target Antibody	GM-Tg-g-T12075-Ab	Anti-SHH/ HHG1/ HLP3 monoclonal antibody
Target Antigen	GM-Tg-g-T12075-Ag	SHH VLP (virus-like particle)
ORF Viral Vector	pGMAAV000001	Human SHH Adeno-associate virus(AAV) plasmid
ORF Viral Vector	pGMLP-SPh-072	Human SHH Lentivirus plasmid
ORF Viral Vector	pGMAP-SPh-212	Human SHH Adenovirus plasmid
ORF Viral Vector	vGMAAV000001	Human SHH Adeno-associate virus(AAV) particle
ORF Viral Vector	vGMLP-SPh-072	Human SHH Lentivirus particle
ORF Viral Vector	vGMAP-SPh-212	Human SHH Adenovirus particle

Target information

Target ID	GM-T12075
Target Name	SHH
Gene ID	6469, 20423, 716553, 29499, 111557492, 608860, 286821, 100147450
Gene Symbol and Synonyms	9530036O11Rik,Dsh,HHG1,HLP3,HPE3,Hx,Hxl3,M100081,MCOPCB5,SHH,ShhNC,SMMCI,TPT,TPTPS
Uniprot Accession	Q15465
Uniprot Entry Name	SHH_HUMAN
Protein Sub-location	Transmembrane Protein
Category	Therapeutics Target
Disease	Not Available
Gene Ensembl	ENSG00000164690
Target Classification	Not Available

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]

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