Human Metabolome Database Version 2.5

Showing metabocard for 3-Hydroxyisovaleryl-CoA (HMDB06870)

Legend: metabolite field enzyme field

Version

2.5

Creation Date

2008-08-13 18:24:17

Update Date

2009-05-05 21:01:17

Accession Number

HMDB06870

Secondary Accession Numbers

Not Available

Common Name

3-Hydroxyisovaleryl-CoA

Description

3-Hydroxyisovaleryl-CoA is an end product of leucine degradation. It is converted from 3-methylbut-2-enoyl-CoA by the enzyme enoyl-CoA hydratase.

Synonyms

3-Hydroxyisovaleryl coenzyme A
3-hydroxy-3-methylbutanoyl-CoA
3-hydroxy-3-methylbutyryl-CoA
3-hydroxy-3-methylbutanoyl-Coenzyme A
3-hydroxy-3-methylbutyryl-Coenzyme A

Chemical IUPAC Name

3'-phosphoadenosine 5'-{3-[(3R)-3-hydroxy-4-{[3-({2-[(3-hydroxy-3-methylbutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-2,2-dimethyl-4-oxobutyl] dihydrogen diphosphate}

Chemical Formula

C₂₆H₄₄N₇O₁₈P₃S

Chemical Structure

Chemical Taxonomy

Kingdom
Organic
Super Class
Nucleosides and Nucleoside conjugates
Class
Coenzyme A Derivatives
Sub Class
Short chain acyl CoAs
Family
Mammalian Metabolite
Species
secondary alcohol tertiary alcohol primary amine primary aromatic amine secondary carboxylic acid amide thiocarboxylic acid ester phosphoric acid ester aromatic compound heterocyclic compound
Biofunction
—
Application
—
Source
Endogenous

Average Molecular Weight

867.650

Monoisotopic Molecular Weight

867.167664

Isomeric SMILES

CC(C)(O)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N

Canonical SMILES

CC(C)(O)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N

KEGG Compound ID

C05998

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

NuGOwiki Link

HMDB06870

Metagene Link

HMDB06870

METLIN ID

Not Available

PubChem Compound

8272

PubChem Substance

Not Available

ChEBI ID

28291

CAS Registry Number

Not Available

InChI Identifier

InChI=1/C26H44N7O18P3S/c1-25(2,20(37)23(38)29-6-5-15(34)28-7-8-55-16(35)9-26(3,4)39)11-48-54(45,46)51-53(43,44)47-10-14-19(50-52(40,41)42)18(36)24(49-14)33-13-32-17-21(27)30-12-31-22(17)33/h12-14,18-20,24,36-37,39H,5-11H2,1-4H3,(H,28,34)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t14-,18-,19-,20?,24-/m1/s1

Synthesis Reference

Not Available

Melting Point (Experimental)

Not Available

Experimental Water Solubility

Not Available Source: PhysProp

Predicted Water Solubility

4.22 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS

Physiological Charge

-4

State

Solid

Experimental LogP/Hydrophobicity

Not Available Source: PhysProp

Predicted LogP/Hydrophobicity

-0.43 [Predicted by ALOGPS] 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
Show Peaklist

Predicted ¹³C NMR Spectrum

Show Image
Show Peaklist

Mass Spectrum

Not Available

Simplified TOCSY Spectrum

Not Available

BMRB Spectrum

Not Available

Cellular Location

Not Available

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
Valine, Leucine and Isoleucine Degradation	SMP00032	map00280

General References

Not Available

Metabolic Enzymes

Enzyme 1 [top]

Enzyme 1 ID

5625

Enzyme 1 Name

Enoyl-CoA hydratase, mitochondrial precursor

Enzyme 1 Synonyms

Short chain enoyl-CoA hydratase
SCEH
Enoyl-CoA hydratase 1

Enzyme 1 Gene Name

ECHS1

Enzyme 1 Protein Sequence

>Enoyl-CoA hydratase, mitochondrial precursor

MAALRVLLSCVRGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNA
LCDGLIDELNQALKTFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHW
DHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLT
RAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMA
KESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ

Enzyme 1 Number of Residues

290

Enzyme 1 Molecular Weight

31388

Enzyme 1 Theoretical pI

8.19

Enzyme 1 GO Classification

Function
catalytic activity
Process
metabolism physiological process
Component
—

Enzyme 1 General Function

Lipid transport and metabolism

Enzyme 1 Specific Function

Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate

Enzyme 1 Pathways

Benzoate Degradation via CoA Ligation (map00632 )
Beta Alanine Metabolism (map00410 )
Butyrate Metabolism (map00650 )
Caprolactam degradation (map00930 )
Fatty Acid Elongation In Mitochondria (map00062 )
Fatty Acid Metabolism (map00071 )
Limonene and pinene degradation (map00903 )
Lysine Degradation (map00310 )
Propanoate Metabolism (map00640 )
Tryptophan Metabolism (map00380 )
Valine, Leucine and Isoleucine Degradation (map00280 )

Enzyme 1 Reactions

(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H2O

Enzyme 1 Pfam Domain Function

ECH (PF00378 )

Enzyme 1 Signals

1-17

Enzyme 1 Transmembrane Regions

Not Available

Enzyme 1 Essentiality

Not Available

Enzyme 1 GenBank ID Protein

433413

Enzyme 1 UniProtKB/Swiss-Prot ID

P30084

Enzyme 1 UniProtKB/Swiss-Prot Entry Name

ECHM_HUMAN Link Image

Enzyme 1 PDB ID

2DUB

Enzyme 1 PDB File

Show

Enzyme 1 3D Structure

Enzyme 1 Cellular Location

Not Available

Enzyme 1 Gene Sequence

>873 bp

ATGGCCGCCCTGCGTGTCCTGCTGTCCTGCGCCCGCGGCCCGCTGAGGCCCCCGGTTCGC
TGTCCCGCCTGGCGTCCCTTCGCCTCGGGTGCTAACTTTGAGTACATCATCGCAGAAAAA
AGAGGGAAGAATAACACCGTGGGGTTGATCCAACTGAACCGCCCCAAGGCCCTCAATGCA
CTTTGCGATGGCCTGATTGACGAGCTCAACCAGGCCCTGAAGATCTTCGAGGAGGACCCG
GCCGTTGGGGGCATTGTCCTCACCGGCGGGGATAAGGCCTTTGCAGCTGGAGCTGATATC
AAGGAAATGCAGAACCTGAGTTTCCAGGACTGTTACTCCAGCAAGTTCTTGAAGCACTGG
GGCCACCTCACCCAGGTCAAGAAGCCAGTCATCGCTGCTGTCAATGGCTATCCGTTTGGC
GGGGGCTGTGAGCTTGCCATGATGTGTGATATCATCTATGCCGGTGAGAAGGCCCAGTTT
GCACAGCCGGAGATCTTAATAGGAACCATCCCAGGTGCAGGCGGCACCCAGAGACTCACC
CGTGCTGTTGGGAAGTCGCTGGAGCTGGAGATGGTCCTCACCGGTGACGCGATCTCAGCC
CAGGACGCCAAGCAAGCAGGTCTTGTCAGCAAGATTTGTCCTGTTGAGACACTGGTGGAA
GAAGCCATCCAGTGTGCAGAAAAAATTGCCAGCAATTCTAAAATTGTAGTAGCGATGGCC
AAAGAATCAGTGAATGCAGCTTTTGAAATGACATTAACAGAAGGAAGTAAGTTGGAGAAG
AAACTCTTTTATTCAACCTTTGCCACTGATGACCGGAAAGAAGGGATGACCGCGTTTGTG
GAAAAGAGAAAGGCCAACTTCAAAGACCAGTGA

Enzyme 1 GenBank Gene ID

Enzyme 1 GeneCard ID

Enzyme 1 GenAtlas ID

Enzyme 1 HGNC ID

Enzyme 1 Chromosome Location

Enzyme 1 Locus

10q26.2-q26.3

Enzyme 1 SNPs

SNPJam Report Link Image

Enzyme 1 General References

Kanazawa M, Ohtake A, Abe H, Yamamoto S, Satoh Y, Takayanagi M, Niimi H, Mori M, Hashimoto T: Molecular cloning and sequence analysis of the cDNA for human mitochondrial short-chain enoyl-CoA hydratase. Enzyme Protein. 1993;47(1):9-13. [PubMed ]
Janssen U, Davis EM, Le Beau MM, Stoffel W: Human mitochondrial enoyl-CoA hydratase gene (ECHS1): structural organization and assignment to chromosome 10q26.2-q26.3. Genomics. 1997 Mar 15;40(3):470-5. [PubMed ]
Hochstrasser DF, Frutiger S, Paquet N, Bairoch A, Ravier F, Pasquali C, Sanchez JC, Tissot JD, Bjellqvist B, Vargas R, et al.: Human liver protein map: a reference database established by microsequencing and gel comparison. Electrophoresis. 1992 Dec;13(12):992-1001. [PubMed ]
Hubbard MJ, McHugh NJ: Human ERp29: isolation, primary structural characterisation and two-dimensional gel mapping. Electrophoresis. 2000 Nov;21(17):3785-96. [PubMed ]

Enzyme 1 Metabolite References

Not Available

Enzyme 2 [top]

Enzyme 2 ID

13010

Enzyme 2 Name

Enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase

Enzyme 2 Synonyms

Enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase, isoform CRA_b
Enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase variant

Enzyme 2 Gene Name

EHHADH

Enzyme 2 Protein Sequence

>Enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase

MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAG
ADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQV
GLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEE
AIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAA
VQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSV
GVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSG
PKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIAS
STDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFV
GNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLT
GPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFL
SRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHK
GGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPS
SKL

Enzyme 2 Number of Residues

723

Enzyme 2 Molecular Weight

79496

Enzyme 2 Theoretical pI

9.54

Enzyme 2 GO Classification

Function
catalytic activity oxidoreductase activity oxidoreductase activity, acting on the CH-NH group of donors oxidoreductase activity, acting on the CH-NH group of donors, NAD or NADP as acceptor
Process
carboxylic acid metabolism cellular metabolism fatty acid metabolism metabolism organic acid metabolism physiological process
Component
—

Enzyme 2 General Function

Lipid transport and metabolism

Enzyme 2 Specific Function

Not Available

Enzyme 2 Pathways

Not Available

Enzyme 2 Reactions

Not Available

Enzyme 2 Pfam Domain Function

3HCDH (PF00725 )
3HCDH_N (PF02737 )
ECH (PF00378 )

Enzyme 2 Signals

None

Enzyme 2 Transmembrane Regions

None

Enzyme 2 Essentiality

Not Available

Enzyme 2 GenBank ID Protein

62021246

Enzyme 2 UniProtKB/Swiss-Prot ID

Q58EZ5

Enzyme 2 UniProtKB/Swiss-Prot Entry Name

Q58EZ5_HUMAN Link Image

Enzyme 2 PDB ID

Not Available

Enzyme 2 Cellular Location

Not Available

Enzyme 2 Gene Sequence

Not Available

Enzyme 2 GenBank Gene ID

Enzyme 2 GeneCard ID

Enzyme 2 GenAtlas ID

Enzyme 2 HGNC ID

Enzyme 2 Chromosome Location

Not Available

Enzyme 2 Locus

Not Available

Enzyme 2 SNPs

SNPJam Report Link Image

Enzyme 2 General References

Strausberg RL, Feingold EA, Grouse LH, Derge JG, Klausner RD, Collins FS, Wagner L, Shenmen CM, Schuler GD, Altschul SF, Zeeberg B, Buetow KH, Schaefer CF, Bhat NK, Hopkins RF, Jordan H, Moore T, Max SI, Wang J, Hsieh F, Diatchenko L, Marusina K, Farmer AA, Rubin GM, Hong L, Stapleton M, Soares MB, Bonaldo MF, Casavant TL, Scheetz TE, Brownstein MJ, Usdin TB, Toshiyuki S, Carninci P, Prange C, Raha SS, Loquellano NA, Peters GJ, Abramson RD, Mullahy SJ, Bosak SA, McEwan PJ, McKernan KJ, Malek JA, Gunaratne PH, Richards S, Worley KC, Hale S, Garcia AM, Gay LJ, Hulyk SW, Villalon DK, Muzny DM, Sodergren EJ, Lu X, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madan A, Young AC, Shevchenko Y, Bouffard GG, Blakesley RW, Touchman JW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Krzywinski MI, Skalska U, Smailus DE, Schnerch A, Schein JE, Jones SJ, Marra MA: Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proc Natl Acad Sci U S A. 2002 Dec 24;99(26):16899-903. Epub 2002 Dec 11. [PubMed ]
Maruyama K, Sugano S: Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides. Gene. 1994 Jan 28;138(1-2):171-4. [PubMed ]
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S: Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library. Gene. 1997 Oct 24;200(1-2):149-56. [PubMed ]

Enzyme 2 Metabolite References

Not Available

Enzyme 3 [top]

Enzyme 3 ID

13022

Enzyme 3 Name

Putative uncharacterized protein

Enzyme 3 Synonyms

Not Available

Enzyme 3 Gene Name

HADHA

Enzyme 3 Protein Sequence

>Putative uncharacterized protein

MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSK
VNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLS
QEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALP
GAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYL
EEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPA
PLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKD
VKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSF
ERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALP
ISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVK
DGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVA
EDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLK
LPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRF
VDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ

Enzyme 3 Number of Residues

763

Enzyme 3 Molecular Weight

83000

Enzyme 3 Theoretical pI

Not Available

Enzyme 3 GO Classification

Not Available

Enzyme 3 General Function

Not Available

Enzyme 3 Specific Function

Not Available

Enzyme 3 Pathways

Not Available

Enzyme 3 Reactions

Not Available

Enzyme 3 Pfam Domain Function

Not Available

Enzyme 3 Signals

None

Enzyme 3 Transmembrane Regions

None

Enzyme 3 Essentiality

Not Available

Enzyme 3 GenBank ID Protein

Not Available

Enzyme 3 UniProtKB/Swiss-Prot ID

B2R7L4

Enzyme 3 UniProtKB/Swiss-Prot Entry Name

B2R7L4_HUMAN Link Image

Enzyme 3 PDB ID

Not Available

Enzyme 3 Cellular Location

Not Available

Enzyme 3 Gene Sequence

Not Available

Enzyme 3 GenBank Gene ID

Not Available

Enzyme 3 GeneCard ID

B2R7L4

Enzyme 3 GenAtlas ID

Not Available

Enzyme 3 HGNC ID

Not Available

Enzyme 3 Chromosome Location

Not Available

Enzyme 3 Locus

Not Available

Enzyme 3 SNPs

SNPJam Report Link Image

Enzyme 3 General References

Not Available

Enzyme 3 Metabolite References

Not Available

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Human Metabolome Database Version 2.5

Showing metabocard for 3-Hydroxyisovaleryl-CoA (HMDB06870)