<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.w3.org/1999/02/22-rdf-syntax-ns#type> <http://www.biopax.org/release/biopax-level3.owl#BiochemicalReaction> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#comment> "Edited: D'Eustachio, P, 2007-04-21 18:31:40"^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#comment> "Authored: D'Eustachio, P, 2007-04-21 18:31:40"^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#comment> "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol and NADPH + H+ react to form 4,4-dimethylcholesta-8(9),24-dien-3beta-ol and NADP+, catalyzed by LBR in the nuclear envelope. LBR protein spans the inner nuclear envelope, has an aminoterminal region with properties of a laminin receptor and a carboxyterminal domain with sequence similarity to sterol delta14-reductases (Holmer et al. 1998). Studies of material from an individual with HEM/Greenberg skeletal dysplasia indicate that LBR catalyzes the sterol delta14-reductase step of cholesterol biosynthesis in vivo. DNA sequencing revealed homozygosity for a mutant LBR allele encoding a truncated protein in the affected individual, and cells from the individual accumulated cholesta-8,14-dien-3beta-ol in culture. Transfection of wild-type LBR into the cultured cells reversed the accumulation of cholesta-8,14-dien-3beta-ol (Waterham et al. 2003). This observation is surprising because a second gene, TM7SF2, encodes an efficient sterol delta14-reductase that is localized to the endoplasmic reticulum whose expression is up-regulated in response to sterol depletion (Bennati et al. 2006). The physiological roles of LBR and TM7SF2 in vivo remain to be determined."^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#comment> "Reviewed: Jassal, B, 2007-04-21 18:33:24"^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#xref> <http://identifiers.org/pubmed/9878250> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#xref> <urn:biopax:UnificationXref:REACTOME+DATABASE+ID_194674> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#xref> <urn:biopax:UnificationXref:REACTOME_REACT_9995_1> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#xref> <http://identifiers.org/pubmed/16784888> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#xref> <http://identifiers.org/pubmed/12618959> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#dataSource> <urn:biopax:Provenance:reactome_hm_41> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#dataSource> <http://www.reactome.org/biopax/48887Provenance1> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#displayName> "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol is reduced to 4,4-dimethylcholesta-8(9),24-dien-3beta-ol [LBR]"^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#name> "4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol + NADPH + H+ => 4,4-dimethylcholesta-8(9),24-dien-3beta-ol + NADP+ [LBR]"^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#eCNumber> "1.3.1.70"^^<http://www.w3.org/2001/XMLSchema#string> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#left> <http://www.reactome.org/biopax/48887PhysicalEntity681> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#left> <http://www.reactome.org/biopax/48887SmallMolecule1446> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#left> <http://www.reactome.org/biopax/48887SmallMolecule1445> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#right> <http://www.reactome.org/biopax/48887SmallMolecule1447> .
<http://www.reactome.org/biopax/48887BiochemicalReaction4293> <http://www.biopax.org/release/biopax-level3.owl#right> <http://www.reactome.org/biopax/48887PhysicalEntity682> .