Human MPI Recombinant Protein (RPPB1976)
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주문- SKU:
- RPPB1976
- Product type:
- Recombinant Protein
- Size:
- 25ug
- Species:
- Human
- Target:
- MPI
- Synonyms:
- Mannose-6-phosphate isomerase
- Synonyms:
- PMI1
- Synonyms:
- CDG1B
- Synonyms:
- Phosphohexomutase
- Source:
- Escherichia Coli
- Uniprot:
- P34949
Description
| Product Name: | Human MPI Recombinant Protein |
| Product Code: | RPPB1976 |
| Size: | 25µg |
| Species: | Human |
| Target: | MPI |
| Synonyms: | Mannose-6-phosphate isomerase, PMI1, CDG1B, Phosphohexomutase, Phosphomannose isomerase, EC 5.3.1.8, FLJ39201. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The MPI solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.4M Urea and 5% glycerol. |
| 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 90% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MAAPRVFPLS CAVQQYAWGK MGSNSEVARL LASSDPLAQI AEDKPYAELW MGTHPRGDAK ILDNRISQKT LSQWIAENQD SLGSKVKDTF NGNLPFLFKV LSVETPLSIQ AHPNKELAEK LHLQAPQHYP DANHKPEMAI ALTPFQGLCG FRPVEEIVTF LKTAAGNNME DIFGELLLQL HQQYPGDIGC FAIYFLNLLT LKPGEAMFLE ANVPHAYLKG DCVECMACSD NTVRAGLTPK FIDVPTLCEM LSYTPSSSKD RLFLPTRSQE DPYLSIYDPP VPDFTIMKTE VPGSVTEYKV LALDSASILL MVQGTVIAST PTTQTPIPLQ RGGVLFIGAN ESVSLKLTEP KDLLIFRACC LL |
MPI is a member of the mannose-6-phosphate isomerase type 1 family. Although MPI is expressed in all tissues, it can be found more abundantly in heart, brain and skeletal muscle. Localized to the cytoplasm, MPI exploits zinc as a cofactor and catalyzes the interconversion of fructose-6-phosphate and mannose-6-phosphate. Mutations in the MPI gene are the cause of carbohydrate-deficient glycoprotein syndrome, type Ib.
MPI Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 382 amino acids (1-362) and having a molecular mass of 41.9 kDa.The MPI is fused to a 20 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | MPI: Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions. Defects in MPI are the cause of congenital disorder of glycosylation type 1B (CDG1B); also known as carbohydrate-deficient glycoprotein syndrome type Ib (CDGS1B). Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under- glycosylated serum glycoproteins. CDG1B is clinically characterized by protein-losing enteropathy. Belongs to the mannose-6-phosphate isomerase type 1 family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Carbohydrate Metabolism - fructose and mannose; Carbohydrate Metabolism - amino sugar and nucleotide sugar; EC 5.3.1.8; Isomerase Chromosomal Location of Human Ortholog: 15q24.1 Cellular Component: cytosol Molecular Function:mannose-6-phosphate isomerase activity Biological Process: GDP-mannose biosynthetic process Disease: Congenital Disorder Of Glycosylation, Type Ib |
| NCBI Summary: | Phosphomannose isomerase catalyzes the interconversion of fructose-6-phosphate and mannose-6-phosphate and plays a critical role in maintaining the supply of D-mannose derivatives, which are required for most glycosylation reactions. Mutations in the MPI gene were found in patients with carbohydrate-deficient glycoprotein syndrome, type Ib. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014] |
| UniProt Code: | P34949 |
| NCBI GenInfo Identifier: | 462567 |
| NCBI Gene ID: | 4351 |
| NCBI Accession: | P34949.2 |
| UniProt Secondary Accession: | P34949,Q96AB0, A8K8K9, |
| UniProt Related Accession: | P34949 |
| Molecular Weight: | 39,834 Da |
| NCBI Full Name: | Mannose-6-phosphate isomerase |
| NCBI Synonym Full Names: | mannose phosphate isomerase |
| NCBI Official Symbol: | MPI |
| NCBI Official Synonym Symbols: | PMI; PMI1; CDG1B |
| NCBI Protein Information: | mannose-6-phosphate isomerase |
| UniProt Protein Name: | Mannose-6-phosphate isomerase |
| UniProt Synonym Protein Names: | Phosphohexomutase; Phosphomannose isomerase; PMI |
| Protein Family: | Mannose-6-phosphate isomerase |
| UniProt Gene Name: | MPI |
| UniProt Entry Name: | MPI_HUMAN |
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