Human BMP13 Recombinant Protein (RPPB0107)
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주문- SKU:
- RPPB0107
- Product type:
- Recombinant Protein
- Size:
- 50ug
- Species:
- Human
- Target:
- BMP13
- Synonyms:
- Growth Differentiation Factor 6
- Synonyms:
- Growth/Differentiation Factor 16
- Synonyms:
- Bone Morphogenetic Protein 13
- Synonyms:
- BMP-13
- Source:
- Escherichia Coli
- Uniprot:
- Q6KF10
Description
| Product Name: | Human BMP13 Recombinant Protein |
| Product Code: | RPPB0107 |
| Size: | 50µg |
| Species: | Human |
| Target: | BMP13 |
| Synonyms: | Growth Differentiation Factor 6, Growth/Differentiation Factor 16, Bone Morphogenetic Protein 13, BMP-13, BMP13, GDF-6, Klippel-Feil Malformation, Segmentation Syndrome 1, Klip-Feil Malformation, Klippel-Feil Syndrome, MCOPCB6, SCDO4, CDMP2, LCA17, MCOP4, GDF16, KFS1, KFSL, SGM1, KFM, KFS, GDF6. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered White lyophilized (freeze-dried) powder. |
| Formulation: | BMP-13 protein was lyophilized from a 0.2µm filtered concentrated solution in 30% Acetonitrile and 0.1% TFA. |
| Solubility: | It is recommended to reconstitute the lyophilized BMP13 in sterile 18M-cm H2O not less than 100µg/ml, which can then be further diluted to other aqueous solutions. |
| Stability: | Lyophilized BMP13 although stable at room temperature for 3 weeks, should be stored desiccated below -18°C. Upon reconstitution BMP-13 should be stored at 4°C between 2-7 days and for future use below -18°C. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Please prevent freeze-thaw cycles. |
| Purity: | Greater than 95.0% as determined by: (a) Analysis by HPLC. (b) Analysis by SDS-PAGE. |
| Amino Acid Sequence: | TAFASRHGKR HGKKSRLRCS KKPLHVNFKE LGWDDWIIAP LEYEAYHCEG VCDFPLRSHL EPTNHAIIQT LMNSMDPGST PPSCCVPTKL TPISILYIDA GNNVVYKQYE DMVVESCGCR |
| Biological Activity: | The ED50 as determined by inducing alkaline phosphatase production of murine ATDC5 cells is less than 2.0µg/ml, corresponding to a specific activity of > 500IU/mg. |
Growth/differentiation factors (GDF1-GDF15) belong to the BMP family of TGF-beta superfamily proteins. These factors are produced as inactive preproproteins which are subsequently cleaved and assembled into active secreted homodimers. BMP13 is a growth factor which controls proliferation and cellular differentiation in the retina and bone formation. BMP13 has a central role in regulating apoptosis during retinal development. GDF proteins are vital during embryonic development, particularly in the skeletal, nervous, and muscular systems. BMP13 gene mutations result in colobomata, which are congenital abnormalities in ocular development, and in Klippel-Feil syndrome (KFS), which is a congenital disorder of spinal segmentation.
BMP13 Human Recombinant produced in E.coli is a non-glycosylated disulfide linked homodimer containing 2 chains of 120 amino acids and having a molecular mass of 27.1kDa.The BMP-13 is purified by proprietary chromatographic techniques.
| UniProt Protein Function: | GDF6: Required for normal formation of bones and joints in the limbs, skull, and axial skeleton. Plays a key role in establishing boundaries between skeletal elements during development. Defects in GDF6 are the cause of Klippel-Feil syndrome type 1 (KFS1). A skeletal disorder characterized by congenital fusion of cervical vertebrae. It is due to a failure in the normal segmentation of vertebrae during the early weeks of fetal development. The clinical triad consists of short neck, low posterior hairline, and limited neck movement. Deafness is a well- known feature of KFS and may be of sensorineural, conductive, or mixed type. A chromosomal aberration involving GDF6 has been found in a patient with Klippel-Feil syndrome (KFS). Paracentric inv(8)(q22;2q23.3). Defects in GDF6 are the cause of microphthalmia isolated type 4 (MCOP4). 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, cataract and other abnormalities like cataract may also be present. Belongs to the TGF-beta family. |
| UniProt Protein Details: | Protein type:Cytokine; Cell development/differentiation; Secreted, signal peptide; Secreted Chromosomal Location of Human Ortholog: 8q22.1 Cellular Component: extracellular space Molecular Function:cytokine activity; growth factor activity; protein homodimerization activity; transforming growth factor beta receptor binding Biological Process: activin receptor signaling pathway; apoptosis; BMP signaling pathway; cell development; growth; positive regulation of neuron differentiation; positive regulation of transcription, DNA-dependent; regulation of apoptosis; regulation of MAPKKK cascade Disease: Klippel-feil Syndrome 1, Autosomal Dominant; Leber Congenital Amaurosis 17; Microphthalmia, Isolated 4; Microphthalmia, Isolated, With Coloboma 6 |
| NCBI Summary: | This gene encodes a member of the bone morphogenetic protein (BMP) family and the TGF-beta superfamily of secreted signaling molecules. It is required for normal formation of some bones and joints in the limbs, skull, and axial skeleton. Mutations in this gene result in colobomata, which are congenital abnormalities in ocular development, and in Klippel-Feil syndrome (KFS), which is a congenital disorder of spinal segmentation. [provided by RefSeq, Jul 2008] |
| UniProt Code: | Q6KF10 |
| NCBI GenInfo Identifier: | 74748876 |
| NCBI Gene ID: | 392255 |
| NCBI Accession: | Q6KF10.1 |
| UniProt Secondary Accession: | Q6KF10,Q6PI58, |
| UniProt Related Accession: | Q6KF10 |
| Molecular Weight: | 50,662 Da |
| NCBI Full Name: | Growth/differentiation factor 6 |
| NCBI Synonym Full Names: | growth differentiation factor 6 |
| NCBI Official Symbol: | GDF6 |
| NCBI Official Synonym Symbols: | KFM; KFS; KFS1; KFSL; SGM1; BMP13; CDMP2; LCA17; MCOP4; SCDO4; BMP-13; MCOPCB6 |
| NCBI Protein Information: | growth/differentiation factor 6 |
| UniProt Protein Name: | Growth/differentiation factor 6 |
| UniProt Synonym Protein Names: | Bone morphogenetic protein 13; BMP-13; Growth/differentiation factor 16 |
| Protein Family: | Growth/differentiation factor |
| UniProt Gene Name: | GDF6 |
| UniProt Entry Name: | GDF6_HUMAN |
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