Description
| 抗体名: | UBIAD1 Antibody (PACO58068) |
| 抗体コード: | PACO58068 |
| サイズ: | 50ug |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, IF |
| 推奨される希釈: | ELISA:1:2000-1:10000, IF:1:50-1:200 |
| 反応性: | Human |
| 免疫原: | Recombinant Human UbiA prenyltransferase domain-containing protein 1 protein (1-82AA) |
| 憲法: | Liquid |
| ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| 精製方法: | >95%, Protein G purified |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
 | Immunofluorescence staining of HepG2 cells with PACO58068 at 1:66, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
| バックグラウンド: | Prenyltransferase that mediates the formation of menaquinone-4 (MK-4) and coenzyme Q10. MK-4 is a vitamin K2 isoform present at high concentrations in the brain, kidney and pancreas, and is required for endothelial cell development. Mediates the conversion of phylloquinone (PK) into MK-4, probably by cleaving the side chain of phylloquinone (PK) to release 2-methyl-1,4-naphthoquinone (menadione; K3) and then prenylating it with geranylgeranyl pyrophosphate (GGPP) to form MK-4. Also plays a role in cardiovascular development independently of MK-4 biosynthesis, by acting as a coenzyme Q10 biosyntetic enzyme: coenzyme Q10, also named ubiquinone, plays a important antioxidant role in the cardiovascular system. Mediates biosynthesis of coenzyme Q10 in the Golgi membrane, leading to protect cardiovascular tissues from NOS3/eNOS-dependent oxidative stress. |
| シノニム: | UbiA prenyltransferase domain-containing protein 1 (EC 2.5.1) (Transitional epithelial response protein 1), UBIAD1, TERE1 |
| UniProt Protein Function: | UBIAD1: Prenyltransferase that mediates the formation of menaquinone-4 (MK-4), a vitamin K2 isoform present at high concentrations in the brain, kidney and pancreas. Mediates the conversion of phylloquinone (PK) into menaquinone-4 (MK-4), probably by cleaving the side chain of phylloquinone (PK) to release 2-methyl-1,4-naphthoquinone (menadione; K3) and then prenylating it with geranylgeranyl pyrophosphate (GGPP) to form MK-4. Defects in UBIAD1 are the cause of crystalline corneal dystrophy of Schnyder (SCCD). SCCD is a rare autosomal dominant disease characterized by progressive corneal opacification resulting from abnormal deposition of cholesterol and phospholipids. Belongs to the UbiA prenyltransferase family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:EC 2.5.1.-; Membrane protein, integral; Membrane protein, multi-pass Chromosomal Location of Human Ortholog: 1p36.22 Cellular Component: cytoplasm; endoplasmic reticulum; endoplasmic reticulum membrane; integral to Golgi membrane; membrane; nucleus Molecular Function:antioxidant activity; prenyltransferase activity; protein binding Biological Process: menaquinone biosynthetic process; ubiquinone biosynthetic process; ubiquinone biosynthetic process via 3,4-dihydroxy-5-polyprenylbenzoate; vitamin K biosynthetic process; vitamin K metabolic process Disease: Schnyder Corneal Dystrophy |
| NCBI Summary: | This gene encodes a protein thought to be involved in cholesterol and phospholipid metabolism. Mutations in this gene are associated with Schnyder crystalline corneal dystrophy. [provided by RefSeq, Oct 2008] |
| UniProt Code: | Q9Y5Z9 |
| NCBI GenInfo Identifier: | 1057866085 |
| NCBI Gene ID: | 29914 |
| NCBI Accession: | |
| UniProt Secondary Accession: | Q9Y5Z9,Q53GX3, Q5THD4, B3KQG3, |
| UniProt Related Accession: | Q9Y5Z9 |
| Molecular Weight: | Calculated MW 19kDa/36kDaObserved MW 37kDa |
| NCBI Full Name: | ubiA prenyltransferase domain-containing protein 1 isoform 2 |
| NCBI Synonym Full Names: | UbiA prenyltransferase domain containing 1 |
| NCBI Official Symbol: | UBIAD1 |
| NCBI Official Synonym Symbols: | SCCD; TERE1 |
| NCBI Protein Information: | ubiA prenyltransferase domain-containing protein 1 |
| UniProt Protein Name: | UbiA prenyltransferase domain-containing protein 1 |
| UniProt Synonym Protein Names: | Transitional epithelial response protein 1 |
| Protein Family: | UbiA prenyltransferase domain-containing protein |
| UniProt Gene Name: | UBIAD1 |
| UniProt Entry Name: | UBIA1_HUMAN |
