Proteins > Peroxisomal multifunctional enzyme 2 (MFE2)
Peroxisomal multifunctional enzyme 2 (MFE2)
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UniProt Number:
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P51659
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Alternate Names:
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D-bifunctional protein, DBP, 17-beta-hydroxysteroid dehydrogenase 4, 17-beta-HSD 4
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Structure and Function:
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MFE2 is involved in the catalysis of the D-stereoisomers of long chain fatty acids (C24 and above) as well as beta-oxidation of branched chain fatty acids such as pristanic acid. Also, MFE2 is involved in the synthesis of bile salts di and trihydroxycholestanoic acid. The protein is around 70kDa and made up of 3 domains, a short chain alcohol dehydrogenase reductase, a central hydratase domain and a C terminal sterol carrier protein 2 domain.
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Disease Associations:
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Defects in MFE2 are a cause of D-bifunctional protein deficiency (DBPD) [MIM:261515]. DBPD is a disorder of peroxisomal fatty acid beta-oxidation.
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Monoclonal Antibodies
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