HSD17B4 Antibody (PACO09810)
- SKU:
- PACO09810
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
| 抗体名: | HSD17B4 Antibody (PACO09810) |
| 抗体コード: | PACO09810 |
| サイズ: | 50ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB, IHC, IF |
| 推奨される希釈: | |
| 反応性: | Human, Mouse, Rat |
| 免疫原: | Human HSD17B4 |
| 憲法: | Liquid |
| ストレージバッファ: | PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
| 精製方法: | Antigen Affinity purified |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
| シノニム: | hydroxysteroid (17-beta) dehydrogenase 4;HSD17B4;DBP;MFE-2;SDR8C1 ; |
| UniProt Protein Function: | HSD17B4: Bifunctional enzyme acting on the peroxisomal beta- oxidation pathway for fatty acids. Catalyzes the formation of 3- ketoacyl-CoA intermediates from both straight-chain and 2-methyl- branched-chain fatty acids. Defects in HSD17B4 are a cause of D-bifunctional protein deficiency (DBPD). DBPD is a disorder of peroxisomal fatty acid beta-oxidation. Defects in HSD17B4 are the cause of Perrault syndrome (PRS). PRS is a sex-influenced disorder characterized by sensorineural deafness in both males and females and ovarian dysgenesis in females. Some patients also have neurologic manifestations, including mild mental retardation and cerebellar and peripheral nervous system involvement. Belongs to the short-chain dehydrogenases/reductases (SDR) family. |
| UniProt Protein Details: | Protein type:EC 4.2.1.107; Lyase; Oxidoreductase; Cell development/differentiation; EC 4.2.1.119; Lipid Metabolism - primary bile acid biosynthesis; EC 1.1.1.n12; Mitochondrial Chromosomal Location of Human Ortholog: 5q21 Cellular Component: intracellular membrane-bound organelle; membrane; peroxisomal matrix; peroxisomal membrane; peroxisome Molecular Function:3-hydroxyacyl-CoA dehydrogenase activity; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity; long-chain-enoyl-CoA hydratase activity; protein homodimerization activity; receptor binding Biological Process: androgen metabolic process; bile acid biosynthetic process; estrogen metabolic process; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA oxidase; osteoblast differentiation Disease: D-bifunctional Protein Deficiency; Perrault Syndrome 1 |
| NCBI Summary: | The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). An apparent pseudogene of this gene is present on chromosome 8. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, May 2014] |
| UniProt Code: | P51659 |
| NCBI GenInfo Identifier: | 1706396 |
| NCBI Gene ID: | 3295 |
| NCBI Accession: | P51659.3 |
| UniProt Secondary Accession: | P51659,B4DNV1, B4DVS5, E9PB82, F5HE57, |
| UniProt Related Accession: | P51659 |
| Molecular Weight: | 77,870 Da |
| NCBI Full Name: | Peroxisomal multifunctional enzyme type 2 |
| NCBI Synonym Full Names: | hydroxysteroid 17-beta dehydrogenase 4 |
| NCBI Official Symbol: | HSD17B4 |
| NCBI Official Synonym Symbols: | DBP; MFE-2; MPF-2; PRLTS1; SDR8C1 |
| NCBI Protein Information: | peroxisomal multifunctional enzyme type 2 |
| UniProt Protein Name: | Peroxisomal multifunctional enzyme type 2 |
| UniProt Synonym Protein Names: | 17-beta-hydroxysteroid dehydrogenase 4; 17-beta-HSD 4 |
| UniProt Gene Name: | HSD17B4 |
| UniProt Entry Name: | DHB4_HUMAN |