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Cytochrome P-450 CYP27A1 (Cholestanetriol 26 Monooxygenase)

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An NAPH-dependent cytochrome P450 enzyme that catalyzes the oxidation of the side chain of sterol intermediates such as the 27-hydroxylation of 5-beta-cholestane-3-alpha,7-alpha, 12-alpha-triol. Cytochrome P-450 CYP27A1 is a mitochondrial enzyme; however microsomal-derived homologs of the enzyme have been identified and are included under this heading.

Also Known As:

Cholestanetriol 26 Monooxygenase; 5-beta-Cholestane-3-alpha,7-alpha,12-alpha-triol 27-Hydroxylase; C27-Steroid 26-Hydroxylase; Cholestanetriol 26-Monooxygenase; Cytochrome P-450 CYP2R1; Cytochrome P-450 Steroid 27-Hydroxylase; Cytochrome P-450 Sterol 26-Hydroxylase; Cytochrome P450 2R1; Cytochrome P450 CYP27; Cytochrome P450, Family 2, Subfamily R, Polypeptide 1; Cytochrome P450, Family 27, Subfamily A, Polypeptide 1; Cytochrome P450c26; Cytochrome P450c26-25; Steroid 25-Hydroxylase; Steroid 27-Hydroxylase; Sterol 26-Hydroxylase; Sterol 27-Hydroxylase; Vitamin D3 25-Hydroxylase; C27 Steroid 26 Hydroxylase; CYP27, Cytochrome P450; CYP27A1, Cytochrome P-450; CYP2R1, Cytochrome P-450; Cytochrome P 450 CYP27A1; Cytochrome P 450 CYP2R1; Cytochrome P 450 Steroid 27 Hydroxylase; Cytochrome P 450 Sterol 26 Hydroxylase; Cytochrome P450c26 25; P-450 CYP27A1, Cytochrome; P-450 CYP2R1, Cytochrome; P450 CYP27, Cytochrome; P450c26-25, Cytochrome; Steroid 25 Hydroxylase; Steroid 27 Hydroxylase; Sterol 26 Hydroxylase; Sterol 27 Hydroxylase; Vitamin D3 25 Hydroxylase

Networked: 96 relevant articles (0 outcomes, 3 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Björkhem, Ingemar: 7 articles (08/2013 - 07/2002)
2.	Leitersdorf, E: 4 articles (09/2001 - 07/2000)
3.	Federico, A: 3 articles (05/2011 - 06/2006)
4.	Dotti, M T: 3 articles (05/2011 - 06/2006)
5.	Gallus, G N: 3 articles (05/2011 - 06/2006)
6.	Hansson, Magnus: 3 articles (09/2010 - 05/2007)
7.	Meiner, Vardiella: 3 articles (09/2010 - 04/2006)
8.	von Bahr, Sara: 3 articles (04/2005 - 07/2002)
9.	Salen, G: 3 articles (03/2002 - 06/2001)
10.	Hannequin, D: 2 articles (06/2014 - 11/2010)

Related Diseases

1.	Cerebrotendinous Xanthomatosis 01/01/2014 - "Cerebrotendinous xanthomatosis (CTX) OMIM#213700 is a rare autosomal-recessive lipid storage disease caused by mutations in the CYP27A1 gene; this gene codes for the mitochondrial enzyme sterol 27-hydroxylase, which is involved in bile acid synthesis. " 03/01/2013 - "Accumulation of 7α-hydroxy-4-cholesten-3-one in patients with lack of sterol 27-hydroxylase (Cerebrotendinous xanthomatosis was shown to be an important pathogenetic factor. " 01/01/2012 - "Cerebrotendinous Xanthomatosis (CTX) is a rare autosomal recessive lipid storage disease caused by sterol 27-hydroxylase (CYP27) deficiency. " 01/01/2012 - "Further genetic analysis revealed homozygozity for mutations in the sterol 27-hydroxylase gene, confirming the diagnosis of cerebrotendinous xanthomatosis (CTX). " 01/01/2011 - "We investigated the clinic manifestation, histopathology and sterol 27-hydroxylase gene (CYP27A1) in a Chinese family with Cerebrotendinous Xanthomatosis (CTX). "
2.	Rickets (Rachitis) 01/01/2013 - "Our study aimed to identify the relationship between three vitamin D-related genes (group specific component [GC], cytochrome P450, family 2, subfamily R, polypeptide 1 (CYP2R1), and 7-dehydrocholesterol reductase/nicotinamide-adenine dinucleotide synthetase 1 (DHCR7/NADSYN1) and rickets in Han Chinese children from northeastern China. "
3.	Atherosclerosis 02/01/2001 - "A potential role for sterol 27-hydroxylase in atherogenesis." 08/30/1994 - "Absence of this defence mechanism may contribute to the premature atherosclerosis known to occur in patients with sterol 27-hydroxylase deficiency (cerebrotendinous xanthomatosis)." 06/01/2001 - "Recent evidence suggests that sterol 27-hydroxylase may play a role in cholesterol homeostasis and affect atherogenesis. " 02/01/2001 - "Genetic heterogeneity resulting in differences in sterol 27-hydroxylase activity between individuals may affect their ability to deal with accumulated cholesterol in the arterial intima, and hence their relative degree of predisposition to atherosclerosis." 08/30/1994 - "Atherosclerosis and sterol 27-hydroxylase: evidence for a role of this enzyme in elimination of cholesterol from human macrophages."
4.	Xanthomatosis (Xanthoma) 07/01/2000 - "Sterol 27-hydroxylase deficiency: a rare cause of xanthomas in normocholesterolemic humans." 03/01/1994 - "The disorder is manifested by extensive nervous system involvement, juvenile cataracts, tendon xanthomas, and premature atherosclerosis and is caused by sterol 27-hydroxylase (EC 1.14.13.15) mutations. " 05/01/2007 - "The most serious consequence of sterol 27-hydroxylase deficiency in humans [cerebrotendinous xanthomatosis (CTX)] is the development of cholestanol-containing brain xanthomas. " 09/01/2010 - "The rare disease cerebrotendinous xanthomatosis (CTX) is due to a lack of sterol 27-hydroxylase (CYP27A1) and is characterized by cholestanol-containing xanthomas in brain and tendons. " 07/01/2002 - "Normolipidemic patients with the rare disease cerebrotendinous xanthomatosis, lacking the enzyme sterol 27-hydroxylase (CYP27A1), develop prominent xanthomas in tendons and brain containing both cholestanol and cholesterol, with a cholestanol:cholesterol ratio higher than that in the circulation. "
5.	Rare Diseases (Rare Disease) 03/01/1994 - "The three sterol 27-hydroxylase gene mutations account for all 10 CTX families and their presence may suggest the existence of positive selective forces that lead to an increased prevalence of this relatively rare disease in Jews from North Africa." 09/01/2010 - "The rare disease cerebrotendinous xanthomatosis (CTX) is due to a lack of sterol 27-hydroxylase (CYP27A1) and is characterized by cholestanol-containing xanthomas in brain and tendons. " 05/21/2010 - "Cerebrotendinous xanthomatosis [CTX] is a rare disease characterized by the accumulation of cholesterol and cholestanol in brain and tendons caused by a mutation in the sterol 27-hydroxylase gene [CYP27A1] involved in bile acid synthesis. " 07/01/2002 - "Normolipidemic patients with the rare disease cerebrotendinous xanthomatosis, lacking the enzyme sterol 27-hydroxylase (CYP27A1), develop prominent xanthomas in tendons and brain containing both cholestanol and cholesterol, with a cholestanol:cholesterol ratio higher than that in the circulation. "

Related Drugs and Biologics

1.	Oxidoreductases
2.	Bile Acids and Salts (Bile Acids)
3.	Vitamin D
4.	NAD (NADH)
5.	Ligases
6.	Cholesterol
7.	Cholestanol (Coprostanol)
8.	Cholesterol 7-alpha-Hydroxylase (Cholesterol 7 alpha Hydroxylase)
9.	Chenodeoxycholic Acid (Chenix)
10.	RNA Splice Sites

Related Therapies and Procedures

1.	Drug Therapy (Chemotherapy)
2.	Chemoprevention