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Human Metabolome Database Version 2.5

 

Showing metabocard for Zymosterol intermediate 2 (HMDB06271)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2007-05-22 21:50:56
Update Date 2009-05-05 21:00:53
Accession Number HMDB06271
Secondary Accession Numbers Not Available
Common Name Zymosterol intermediate 2
Description Zymosterol is the precursor of cholesterol and is found in the plasma membrane. zymosterol circulates within the cells. The structural features of zymosterol provided optimal substrate acceptability. In human fibroblasts, zymosterol is converted to cholesterol solely in the rough ER. Little or no zymosterol or cholesterol accumulates in the rough ER in vivo. Newly synthesized zymosterol moves to the plasma membrane without a detectable lag and with a half-time of 9 min, about twice as fast as cholesterol. The pool of radiolabeled zymosterol in the plasma membrane turns over rapidly, faster than does intracellular cholesterol. Thus, plasma membrane zymosterol is not stagnant. [3H]Zymosterol pulsed into intact cells is initially found in the plasma membrane. (PMID: 1939176)
Synonyms
  1. delta8,24-Cholestadien-3beta-ol
  2. Cholesta-8,24-dien-3-ol
  3. Zymosterol
  4. 5alpha-Cholesta-8,24-dien-3beta-ol
  5. Cholest-8,24-dien-3b-ol
  6. (3b,5a)- Cholesta-8,24-dien-3-ol
Chemical IUPAC Name (3S,5S,10S,13R,14R,17R)-10,13-dimethyl-17-[(2R)-6-methylhept-5-en-2-yl]-2,3,4,5,6,7,11,12,14,15,16,17-dodecahydro-1H-cyclopenta[a]phenanthren-3-ol
Chemical Formula C27H44O
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Cholesterols and derivatives
Class
  • Steroids and Steroid Derivatives
Sub Class
  • Miscellaneous steroids
Family
  • Mammalian Metabolite
Species
  • secondary alcohol
  • alkene
Biofunction
  • Hormones, Membrane component
Application
—
Source
  • Endogenous
Average Molecular Weight 384.638
Monoisotopic Molecular Weight 384.339203
Isomeric SMILES C[C@H](CCC=C(C)C)[C@H]1CC[C@H]2C3=C(CC[C@]12C)[C@@]1(C)CC[C@H](O)C[C@@H]1CC3
Canonical SMILES CC(CCC=C(C)C)C1CCC2C3=C(CCC12C)C1(C)CCC(O)CC1CC3
KEGG Compound ID C05437 Link Image
BioCyc ID Not Available
BiGG ID 1454806 Link Image
Wikipedia Link Not Available
NuGOwiki Link HMDB06271 Link Image
Metagene Link HMDB06271 Link Image
METLIN ID Not Available
PubChem Compound 92746 Link Image
PubChem Substance 8143834 Link Image
ChEBI ID 18252 Link Image
CAS Registry Number 128-33-6
InChI Identifier InChI=1/C27H44O/c1-18(2)7-6-8-19(3)23-11-12-24-22-10-9-20-17-21(28)13-15-26(20,4)25(22)14-16-27(23,24)5/h7,19-21,23-24,28H,6,8-17H2,1-5H3/t19-,20+,21+,23-,24+,26+,27-/m1/s1
Synthesis Reference Dolle, Roland E.; Schmidt, Stanley J.; Kruse, Lawrence I. Synthesis of zymosterol: salient intermediate of fungal and mammalian sterol biosynthesis. Journal of the Chemical Society, Chemical Communications (1988), (1), 19-21.
Melting Point (Experimental) 110 oC
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 2.54e-07 mg/mL at 25 oC [MEYLAN,WM et al. (1996)]; 9.98e-04 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge 0
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity 6.8 [Predicted by PubChem via XLOGP]; 6.51 [Predicted by ALOGPS]; 8.65 [MEYLAN,WM & HOWARD,PH (1995)] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
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Predicted 13C NMR Spectrum Show Image
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Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • endoplasmic reticulum
Biofluid Location Not Available
Tissue Location Not Available
Concentrations (Normal) Not Available
Concentrations (Abnormal) Not Available
Associated Disorders Not Available
OMIM ID Not Available
Pathways
Name SMPDB Link KEGG Link
Steroid Biosynthesis SMP00023 Link Image map00100 Link Image
General References Not Available
Metabolic Enzymes
  1. 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase
  2. 24-dehydrocholesterol reductase precursor
Enzyme 1 [top]
Enzyme 1 ID 6237
Enzyme 1 Name 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase
Enzyme 1 Synonyms
  1. Cholestenol Delta-isomerase
  2. Delta(8-Delta(7sterol isomerase
  3. D8-D7 sterol isomerase
  4. Emopamil-binding protein
Enzyme 1 Gene Name EBP
Enzyme 1 Protein Sequence >3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase 
MTTNAGPLHPYWPQHLRLDNFVPNDRPTWHILAGLFSVTGVLVVTTWLLSGRAAVVPLGT
WRRLSLCWFAVCGFIHLVIEGWFVLYYEDLLGDQAFLSQLWKEYAKGDSRYILGDNFTVC
METITACLWGPLSLWVVIAFLRQHPLRFILQLVVSVGQIYGDVLYFLTEHRDGFQHGELG
HPLYFWFYFVFMNALWLVLPGVLVLDAVKHLTHAQSTLDAKATKAKSKKN
Enzyme 1 Number of Residues 230
Enzyme 1 Molecular Weight 26353
Enzyme 1 Theoretical pI 8.02
Enzyme 1 GO Classification
Function
  • catalytic activity
  • cholestenol delta-isomerase activity
  • intramolecular oxidoreductase activity
  • intramolecular oxidoreductase activity, transposing C=C bonds
  • isomerase activity
Process
  • alcohol metabolism
  • cellular metabolism
  • metabolism
  • physiological process
  • sterol metabolism
Component
  • cell
  • endoplasmic reticulum
  • integral to membrane
  • intracellular membrane-bound organelle
  • intrinsic to membrane
  • membrane
  • membrane-bound organelle
  • organelle
Enzyme 1 General Function Not Available
Enzyme 1 Specific Function Catalyzes the conversion of Delta(8)-sterols to their corresponding Delta(7)-isomers
Enzyme 1 Pathways
Enzyme 1 Reactions
  • 5alpha-cholest-7-en-3beta-ol = 5alpha-cholest-8-en-3beta-ol
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • 29-49 66-86 121-141 185-205
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 780263 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q15125 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name EBP_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >693 bp 
ATGACTACCAACGCGGGCCCCTTGCACCCATACTGGCCTCAGCACCTAAGACTGGACAAC
TTTGTACCTAATGACCGCCCCACCTGGCATATACTGGCTGGCCTCTTCTCTGTCACAGGG
GTCTTAGTCGTGACCACATGGCTGTTGTCAGGTCGTGCTGCGGTTGTCCCATTGGGGACT
TGGCGGCGACTGTCCCTGTGCTGGTTTGCAGTGTGTGGGTTCATTCACCTGGTGATCGAG
GGCTGGTTCGTTCTCTACTACGAAGACCTGCTTGGAGACCAAGCCTTCTTATCTCAACTC
TGGAAAGAGTATGCCAAGGGAGACAGCCGATACATCCTGGGTGACAACTTCACAGTGTGC
ATGGAAACCATCACAGCTTGCCTGTGGGGACCACTCAGCCTGTGGGTGGTGATCGCCTTT
CTCCGCCAGCATCCCCTCCGCTTCATTCTACAGCTTGTGGTCTCTGTGGGCCAGATCTAT
GGGGATGTGCTCTACTTCCTGACAGAGCACCGCGACGGATTCCAGCACGGAGAGCTGGGC
CACCCTCTCTACTTCTGGTTTTACTTTGTCTTCATGAATGCCCTGTGGCTGGTGCTGCCT
GGAGTCCTTGTGCTTGATGCTGTGAAGCACCTCACTCATGCCCAGAGCACGCTGGATGCC
AAGGCCACAAAAGCCAAGAGCAAGAAGAACTGA
Enzyme 1 GenBank Gene ID Z37986 Link Image
Enzyme 1 GeneCard ID EBP Link Image
Enzyme 1 GenAtlas ID EBP Link Image
Enzyme 1 HGNC ID HGNC:3133 Link Image
Enzyme 1 Chromosome Location X
Enzyme 1 Locus Xp11.23-p11.22
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Hanner M, Moebius FF, Weber F, Grabner M, Striessnig J, Glossmann H: Phenylalkylamine Ca2+ antagonist binding protein. Molecular cloning, tissue distribution, and heterologous expression. J Biol Chem. 1995 Mar 31;270(13):7551-7. [PubMed Link Image]
  2. Derry JM, Gormally E, Means GD, Zhao W, Meindl A, Kelley RI, Boyd Y, Herman GE: Mutations in a delta 8-delta 7 sterol isomerase in the tattered mouse and X-linked dominant chondrodysplasia punctata. jderry@immunex.com. Nat Genet. 1999 Jul;22(3):286-90. [PubMed Link Image]
  3. Braverman N, Lin P, Moebius FF, Obie C, Moser A, Glossmann H, Wilcox WR, Rimoin DL, Smith M, Kratz L, Kelley RI, Valle D: Mutations in the gene encoding 3 beta-hydroxysteroid-delta 8, delta 7-isomerase cause X-linked dominant Conradi-Hunermann syndrome. Nat Genet. 1999 Jul;22(3):291-4. [PubMed Link Image]
  4. Has C, Bruckner-Tuderman L, Muller D, Floeth M, Folkers E, Donnai D, Traupe H: The Conradi-Hunermann-Happle syndrome (CDPX2) and emopamil binding protein: novel mutations, and somatic and gonadal mosaicism. Hum Mol Genet. 2000 Aug 12;9(13):1951-5. [PubMed Link Image]
  5. Becker K, Csikos M, Horvath A, Karpati S: Identification of a novel mutation in 3beta-hydroxysteroid-Delta8-Delta7-isomerase in a case of Conradi-Hunermann-Happle syndrome. Exp Dermatol. 2001 Aug;10(4):286-9. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 7345
Enzyme 2 Name 24-dehydrocholesterol reductase precursor
Enzyme 2 Synonyms
  1. 3-beta- hydroxysterol delta-24-reductase
  2. Seladin-1
  3. Diminuto/dwarf1 homolog
Enzyme 2 Gene Name DHCR24
Enzyme 2 Protein Sequence >24-dehydrocholesterol reductase precursor 
MEPAVSLAVCALLFLLWVRLKGLEFVLIHQRWVFVCLFLLPLSLIFDIYYYVRAWVVFKL
SSAPRLHEQRVRDIQKQVREWKEQGSKTFMCTGRPGWLTVSLRVGKYKKTHKNIMINLMD
ILEVDTKKQIVRVEPLVTMGQVTALLTSIGWTLPVLPELDDLTVGGLIMGTGIESSSHKY
GLFQHICTAYELVLADGSFVRCTPSENSDLFYAVPWSCGTLGFLVAAEIRIIPAKKYVKL
RFEPVRGLEAICAKFTHESQRQENHFVEGLLYSLDEAVIMTGVMTDEAEPSKLNSIGNYY
KPWFFKHVENYLKTNREGLEYIPLRHYYHRHTRSIFWELQDIIPFGNNPIFRYLFGWMVP
PKISLLKLTQGETLRKLYEQHHVVQDMLVPMKCLQQALHTFQNDIHVYPIWLCPFILPSQ
PGLVHPKGNEAELYIDIGAYGEPRVKHFEARSCMRQLEKFVRSVHGFQMLYADCYMNREE
FWEMFDGSLYHKLREKLGCQDAFPEVYDKICKAARH
Enzyme 2 Number of Residues 516
Enzyme 2 Molecular Weight 60102
Enzyme 2 Theoretical pI 8.24
Enzyme 2 GO Classification
Function
—
Process
  • cellular metabolism
  • electron transport
  • generation of precursor metabolites and energy
  • metabolism
  • physiological process
Component
—
Enzyme 2 General Function Energy production and conversion
Enzyme 2 Specific Function Catalyzes the reduction of the delta-24 double bond of sterol intermediates. Protects cells from oxidative stress by reducing caspase 3 activity during apoptosis induced by oxidative stress. Also protects against amyloid-beta peptide-induced apoptosis
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • 1-22
Enzyme 2 Transmembrane Regions
  • 32-52
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 10442025 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q15392 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name DHC24_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence >1551 bp 
ATGGAGCCCGCCGTGTCGCTGGCCGTGTGCGCGCTGCTCTTCCTGCTGTGGGTGCGCCTG
AAGGGGCTGGAGTTCGTGCTCATCCACCAGCGCTGGGTGTTCGTGTGCCTCTTCCTCCTG
CCGCTCTCGCTTATCTTCGATATCTACTACTACGTGCGCGCCTGGGTGGTGTTCAAGCTC
AGCAGCGCTCCGCGCCTGCACGAGCAGCGCGTGCGGGACATCCAGAAGCAGGTGCGGGAA
TGGAAGGAGCAGGGTAGCAAGACCTTCATGTGCACGGGGCGCCCTGGCTGGCTCACTGTC
TCACTACGTGTCGGGAAGTACAAGAAGACACACAAAAACATCATGATCAACCTGATGGAC
ATTCTGGAAGTGGACACCAAGAAACAGATTGTCCGTGTGGAGCCCTTGGTGACCATGGGC
CAGGTGACTGCCCTGCTGACCTCCATTGGCTGGACTCTCCCCGTGTTGCCTGAGCTTGAT
GACCTCACAGTGGGGGGCTTGATCATGGGCACAGGCATCGAGTCATCATCCCACAAGTAC
GGCCTGTTCCAACACATCTGCACTGCTTACGAGCTGGTCCTGGCTGATGGCAGCTTTGTG
CGATGCACTCCGTCCGAAAACTCAGACCTGTTCTATGCCGTACCCTGGTCCTGTGGGACG
CTGGGTTTCCTGGTGGCCGCTGAGATCCGCATCATCCCTGCCAAGAAGTACGTCAAGCTG
CGTTTCGAGCCAGTGCGGGGCCTGGAGGCTATCTGTGCCAAGTTCACCCACGAGTCCCAG
CGGCAGGAGAACCACTTCGTGGAAGGGCTGCTCTACTCCCTGGATGAGGCTGTCATTATG
ACAGGGGTCATGACAGATGAGGCAGAGCCCAGCAAGCTGAATAGCATTGGCAATTACTAC
AAGCCGTGGTTCTTTAAGCATGTGGAGAACTATCTGAAGACAAACCGAGAGGGCCTGGAG
TACATTCCCTTGAGACACTACTACCACCGCCACACGCGCAGCATCTTCTGGGAGCTCCAG
GACATCATCCCCTTTGGCAACAACCCCATCTTCCGCTACCTCTTTGGCTGGATGGTGCCT
CCCAAGATCTCCCTCCTGAAGCTGACCCAGGGTGAGACCCTGCGCAAGCTGTACGAGCAG
CACCACGTGGTGCAGGACATGCTGGTGCCCATGAAGTGCCTGCAGCAGGCCCTGCACACC
TTCCAAAACGACATCCACGTCTACCCCATCTGGCTGTGTCCGTTCATCCTGCCCAGCCAG
CCAGGCCTAGTGCACCCCAAAGGAAATGAGGCAGAGCTCTACATCGACATTGGAGCATAT
GGGGAGCCGCGTGTGAAACACTTTGAAGCCAGGTCCTGCATGAGGCAGCTGGAGAAGTTT
GTCCGCAGCGTGCATGGCTTCCAGATGCTGTATGCCGACTGCTACATGAACCGGGAGGAG
TTCTGGGAGATGTTTGATGGCTCCTTGTACCACAAGCTGCGAGAGAAGCTGGGTTGCCAG
GACGCCTTCCCCGAGGTGTACGACAAGATCTGCAAGGCCGCCAGGCACTGA
Enzyme 2 GenBank Gene ID AF261758 Link Image
Enzyme 2 GeneCard ID DHCR24 Link Image
Enzyme 2 GenAtlas ID DHCR24 Link Image
Enzyme 2 HGNC ID HGNC:2859 Link Image
Enzyme 2 Chromosome Location 1
Enzyme 2 Locus 1p33-p31.1
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References
  1. Greeve I, Hermans-Borgmeyer I, Brellinger C, Kasper D, Gomez-Isla T, Behl C, Levkau B, Nitsch RM: The human DIMINUTO/DWARF1 homolog seladin-1 confers resistance to Alzheimer's disease-associated neurodegeneration and oxidative stress. J Neurosci. 2000 Oct 1;20(19):7345-52. [PubMed Link Image]
  2. Waterham HR, Koster J, Romeijn GJ, Hennekam RC, Vreken P, Andersson HC, FitzPatrick DR, Kelley RI, Wanders RJ: Mutations in the 3beta-hydroxysterol Delta24-reductase gene cause desmosterolosis, an autosomal recessive disorder of cholesterol biosynthesis. Am J Hum Genet. 2001 Oct;69(4):685-94. Epub 2001 Aug 22. [PubMed Link Image]
  3. Nomura N, Miyajima N, Sazuka T, Tanaka A, Kawarabayasi Y, Sato S, Nagase T, Seki N, Ishikawa K, Tabata S: Prediction of the coding sequences of unidentified human genes. I. The coding sequences of 40 new genes (KIAA0001-KIAA0040) deduced by analysis of randomly sampled cDNA clones from human immature myeloid cell line KG-1. DNA Res. 1994;1(1):27-35. [PubMed Link Image]
Enzyme 2 Metabolite References Not Available