Home > Product > Antibody > Rabbit Anti-HSD17B4/FITC Conjugated antibody
hydroxysteroid (17-beta) dehydrogenase 4; 12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA hydratase; 17 beta HSD 4; 17 beta HSD IV; 17 beta hydroxysteroid dehydrogenase 4; 17-beta-HSD 4; 17-beta-hydroxysteroid dehydrogenase 4; 17beta estradiol dehydrogena
Cat:
SL11296R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Chicken,Pig,Cow,Horse,Sheep,Guinea Pig,Zebrafish)
Immunogen:
KLH conjugated synthetic peptide derived from human HSD17B4 Enoyl-CoA hydratase 2
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
47/80kDa
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Unit:
Price: $
Product PDFs
Datasheet:


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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