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Rabbit Anti-HSD17B4/FITC Conjugated antibody
background:
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.
Function:
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.
Subcellular Location:
Peroxisome.
Tissue Specificity:
Present in many tissues with highest concentrations in liver, heart, prostate and testis.
DISEASE:
Defects in HSD17B4 are a cause of D-bifunctional protein deficiency (DBPD) [MIM:261515]. DBPD is a disorder of peroxisomal fatty acid beta-oxidation.
Similarity:
Belongs to the short-chain dehydrogenases/reductases (SDR) family.
Contains 1 MaoSLClike domain.
Contains 1 SCP2 domain.
Database links:
Entrez Gene: 3295 Human
Entrez Gene: 15488 Mouse
Entrez Gene: 79244 Rat
Omim: 601860 Human
SwissProt: P51659 Human
SwissProt: P51660 Mouse
SwissProt: P97852 Rat
Unigene: 406861 Human
Unigene: 277857 Mouse
Unigene: 2082 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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