羟基类固醇(17β)脱氢酶4/17β-HSD4抗体
产品名称: 羟基类固醇(17β)脱氢酶4/17β-HSD4抗体
英文名称: HSD17B4
产品编号: bs-11296R
产品价格: null
产品产地: 北京
品牌商标:
更新时间: 2025-01-08T10:30:41
使用范围: WB=1:100-500,ELISA=1:500-1000,IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,
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17Beta-HSD4 (17Beta-hydroxysteroid dehydrogenase type 4) is also known as peroxisomal multifunctional enzyme/protein 2 (MFE-2/MFP-2), D-bifunctional enzyme or 17-Beta Estradiol dehydrogenase type IV. It belongs to the 17Beta-HSD family of proteins that regulate the availability of steroids within various tissues throughout the body. 17Beta-HSD4 inactivates Estradiol through its oxidative activity but it is primarily involved in peroxisomal fatty acid and cholesterol Beta-oxidation. It has a multi-domain structure: the dehydrogenase domain is fused to a hydratase and a lipid transfer domain. 17Beta-HSD4 is a target protein of chromeceptin and it is essential for the downstream activation of Stat6. 17Beta-HSD4-deficient patients exhibit Zellweger-like syndrome and die within the first year of life. They display neuronal migration defects, facial dysmorphisms, severe hypotonia and convulsions in the neonatal period.