Human IVD Recombinant Protein (RPPB1853)
- SKU:
- RPPB1853
- Product type:
- Recombinant Protein
- Size:
- 25ug
- Species:
- Human
- Target:
- IVD
- Synonyms:
- FLJ12715
- isovaleryl-CoA dehydrogenase mitochondrial
- FLJ34849
- EC 139910
- Source:
- Escherichia Coli
- Uniprot:
- P26440
Description
| Product Name: | Human IVD Recombinant Protein |
| Product Code: | RPPB1853 |
| Size: | 25µg |
| Species: | Human |
| Target: | IVD |
| Synonyms: | FLJ12715, isovaleryl-CoA dehydrogenase mitochondrial, FLJ34849, EC 1.3.99.10, IVD, ACAD2. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The IVD protein solution (1mg/ml) contains 20mM Tris-HCl pH-8, 1mM DTT and 10% 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 90.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MHSLLPVDDA INGLSEEQRQ LRQTMAKFLQ EHLAPKAQEI DRSNEFKNLR EFWKQLGNLG VLGITAPVQY GGSGLGYLEH VLVMEEISRA SGAVGLSYGA HSNLCINQLV RNGNEAQKEK YLPKLISGEY IGALAMSEPN AGSDVVSMKL KAEKKGNHYI LNGNKFWITN GPDADVLIVY AKTDLAAVPA SRGITAFIVE KGMPGFSTSK KLDKLGMRGS NTCELIFEDC KIPAANILGH ENKGVYVLMS GLDLERLVLA GGPLGLMQAV LDHTIPYLHV REAFGQKIGH FQLMQGKMAD MYTRLMACRQ YVYNVAKACD EGHCTAKDCA GVILYSAECA TQVALDGIQC FGGNGYINDF PMGRFLRDAK LYEIGAGTSE VRRLVIGRAF NADFH |
IVD is a mitochondrial matrix enzyme that is part of the cyl-CoA dehydrogenase family which catalyzes the third step in leucine catabolism. The genetic deficiency of IVD leads to a buildup of isovaleric acid, which is toxic to the central nervous system and results in isovaleric acidemia. IVD is a homotetrameric flavoenzyme which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA.
IVD Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 415 amino acids (33-426 a.a.) and having a molecular mass of 45.3 kDa. The IVD is fused to a 20 amino acid his tag at N-terminus and purified by conventional chromatography.
| UniProt Protein Function: | IVD: Defects in IVD are the cause of isovaleric acidemia (IVA). IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death. Belongs to the acyl-CoA dehydrogenase family. |
| UniProt Protein Details: | Protein type:EC 1.3.8.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 15q14-q15 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix Molecular Function:isovaleryl-CoA dehydrogenase activity; FAD binding Biological Process: leucine catabolic process; branched chain family amino acid catabolic process Disease: Isovaleric Acidemia |
| NCBI Summary: | Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009] |
| UniProt Code: | P26440 |
| NCBI GenInfo Identifier: | 226958414 |
| NCBI Gene ID: | 3712 |
| NCBI Accession: | NP_001152980.1 |
| UniProt Secondary Accession: | P26440,Q53XZ9, Q96AF6, B2RCV5, B3KVI7, J3KR54, |
| UniProt Related Accession: | P26440 |
| Molecular Weight: | 46,319 Da |
| NCBI Full Name: | isovaleryl-CoA dehydrogenase, mitochondrial isoform 2 |
| NCBI Synonym Full Names: | isovaleryl-CoA dehydrogenase |
| NCBI Official Symbol: | IVD |
| NCBI Official Synonym Symbols: | ACAD2 |
| NCBI Protein Information: | isovaleryl-CoA dehydrogenase, mitochondrial; isovaleryl CoA dehydrogenase; isovaleryl Coenzyme A dehydrogenase |
| UniProt Protein Name: | Isovaleryl-CoA dehydrogenase, mitochondrial |
| Protein Family: | Isovaleryl-CoA dehydrogenase |
| UniProt Gene Name: | IVD |
| UniProt Entry Name: | IVD_HUMAN |
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