Information on EC 6.2.1.2 - medium-chain acyl-CoA ligase and Organism(s) Homo sapiens

Reaction Schemes

hideshow

ATP

+

a medium-chain fatty acid

+

CoA

=

AMP

+

diphosphate

+

a medium-chain acyl-CoA

+

+

=

+

+

Synonyms
elongase, macs2, mig protein, medium chain acyl-coa synthetase, butyryl-coa synthetase, xm-ligase, short-chain acyl-coa synthetase, xenobiotic/medium-chain fatty acid:coa ligase, medium chain acyl-coenzyme a synthetase, butyryl-coenzyme a synthetase, more

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SYNONYM

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

Acyl-activating enzyme

-

-

-

-

butanoate:CoA ligase (AMP-forming)

-

-

-

-

butyrate-CoA ligase

-

-

-

-

Butyryl-CoA synthetase

-

-

-

-

Butyryl-coenzyme A synthetase

-

-

-

-

Fatty acid thiokinase (medium chain)

-

-

-

-

L-(+)-3-Hydroxybutyryl CoA ligase

-

-

-

-

MACS2

Homo sapiens

-

-

676089

Medium chain acyl-coenzyme A synthetase

-

-

-

-

medium-chain acyl-CoA synthetase 2

Homo sapiens

-

-

676089

Short-chain acyl-CoA synthetase

-

-

-

-

Synthetase, butyryl conzyme A

-

-

-

-

xenobiotic/medium-chain fatty acid:CoA ligase

Homo sapiens

-

-

651834

XM-ligase

Homo sapiens

-

-

651834

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REACTION TYPE

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

Acid-thiol ligation

-

-

-

-

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PATHWAY SOURCE

PATHWAYS

BRENDA

lipid metabolism

KEGG

Butanoate metabolism

MetaCyc

(3R)-N-[(2S)-1-hydroxy-6-[(3R)-3-isocyanobutanamido]hexan-2-yl]-3-isocyanobutanamide biosynthesis, 2-methyl-branched fatty acid beta-oxidation, 4-oxopentanoate degradation, 6-gingerol analog biosynthesis (engineered), cannabinoid biosynthesis, fatty acid salvage, octane oxidation, palmitate biosynthesis II (type II fatty acid synthase), sporopollenin precursors biosynthesis, valproate beta-oxidation

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SYSTEMATIC NAME

IUBMB Comments

medium-chain fatty acid:CoA ligase (AMP-forming)

Acts on fatty acids from C4 to C11 and on the corresponding 3-hydroxy and 2,3- or 3,4-unsaturated acids. The enzyme from the bacterium Pseudomonas putida also acts on 4-oxo and 4-hydroxy derivatives.

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CAS REGISTRY NUMBER

COMMENTARY

9080-51-7

-

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SUBSTRATE

PRODUCT

REACTION DIAGRAM

ORGANISM

UNIPROT

LITERATURE

COMMENTARY

Reversibility
r=reversible
ir=irreversible
?=not specified

ATP + benzoate + CoA

AMP + diphosphate + benzoyl-CoA

Homo sapiens

-

651834

Substrates: -
Products: -

?

ATP + phenylacetate + CoA

AMP + diphosphate + phenylacetyl-CoA

Homo sapiens

-

651834

Substrates: -
Products: -

?

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DISEASE

TITLE OF PUBLICATION

LINK TO PUBMED

Adenocarcinoma

An evaluation of carcinogenicity predictors from short-term and sub chronic repeat-dose studies of agrochemicals in rats: Opportunities to refine and reduce animal use.

34364947

Adrenoleukodystrophy

ABC Subfamily D Proteins and Very Long Chain Fatty Acid Metabolism as Novel Targets in Adrenoleukodystrophy.

21039332

Breast Neoplasms

Plasmalogen Deficiency and Overactive Fatty Acid Elongation Biomarkers in Serum of Breast Cancer Patients Pre- and Post-Surgery-New Insights on Diagnosis, Risk Assessment, and Disease Mechanisms.

34439324

Cardiovascular Diseases

Elovl6: a new player in fatty acid metabolism and insulin sensitivity.

19259639

Colitis, Ulcerative

Mucosal enzyme activity for butyrate oxidation; no defect in patients with ulcerative colitis.

8984028

Cystic Fibrosis

Increased elongase 6 and ?9-desaturase activity are associated with n-7 and n-9 fatty acid changes in cystic fibrosis.

21544602

Dehydration

Purification of the acyl-CoA elongase complex from developing rapeseed and characterization of the 3-ketoacyl-CoA synthase and the 3-hydroxyacyl-CoA dehydratase.

10907783

Dehydration

The Saccharomyces cerevisiae YBR159w gene encodes the 3-ketoreductase of the microsomal fatty acid elongase.

12087109

Dermatitis, Atopic

Gene expression of desaturase (FADS1 and FADS2) and Elongase (ELOVL5) enzymes in peripheral blood: association with polyunsaturated fatty acid levels and atopic eczema in 4-year-old children.

24167612

Diabetes Mellitus, Type 2

Plasma fatty acids as predictors of glycaemia and type 2 diabetes.

26277381

Epilepsy

Plasma fatty acid abnormality in Sudanese drug-resistant epileptic patients.

33798873

Hepatitis B

Serum lipids as an indicator for the alteration of liver function in patients with hepatitis B.

29506525

Ichthyosis

Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases.

22814679

Infectious Mononucleosis

Interleukin-18, interferon-gamma, IP-10, and Mig expression in Epstein-Barr virus-induced infectious mononucleosis and posttransplant lymphoproliferative disease.

10393857

Insulin Resistance

Associations Among Fatty Acids, Desaturase and Elongase, and Insulin Resistance in Children.

29043930

Insulin Resistance

Crucial role of a long-chain fatty acid elongase, Elovl6, in obesity-induced insulin resistance.

17906635

Insulin Resistance

Elovl6: a new player in fatty acid metabolism and insulin sensitivity.

19259639

Insulin Resistance

Lower estimates of delta-5 desaturase and elongase activity are related to adverse profiles for several metabolic risk factors in young Japanese women.

19083494

Intellectual Disability

Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases.

22814679

Liver Cirrhosis

Advanced Liver Fibrosis Is Independently Associated with Palmitic Acid and Insulin Levels in Patients with Non-Alcoholic Fatty Liver Disease.

30380656

long-chain-aldehyde dehydrogenase deficiency

Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases.

22814679

Macular Degeneration

A Stargardt disease-3 mutation in the mouse Elovl4 gene causes retinal deficiency of C32-C36 acyl phosphatidylcholines.

17983602

Macular Degeneration

ELOVL4 protein preferentially elongates 20:5n3 to very long chain PUFAs over 20:4n6 and 22:6n3.

22158834

Macular Degeneration

Examination of VLC-PUFA-deficient photoreceptor terminals.

24764063

Macular Degeneration

Hetero-oligomeric interactions of an ELOVL4 mutant protein: implications in the molecular mechanism of Stargardt-3 macular dystrophy.

21139992

Malnutrition

Lipids, lipoproteins, and fatty acids during infantile marasmus in the Fès area of Morocco.

10424250

Metabolic Diseases

Associations Among Fatty Acids, Desaturase and Elongase, and Insulin Resistance in Children.

29043930

Metabolic Diseases

Elovl6: a new player in fatty acid metabolism and insulin sensitivity.

19259639

Metabolic Syndrome

Plasma fatty acids as predictors of glycaemia and type 2 diabetes.

26277381

Metabolic Syndrome

Plasma fatty acids profile and estimated elongase and desaturases activities in Tunisian patients with the metabolic syndrome.

21782403

Metabolic Syndrome

The L513S polymorphism in medium-chain acyl-CoA synthetase 2 (MACS2) is associated with risk factors of the metabolic syndrome in a Caucasian study population.

16521160

Neoplasms

An evaluation of carcinogenicity predictors from short-term and sub chronic repeat-dose studies of agrochemicals in rats: Opportunities to refine and reduce animal use.

34364947

Obesity

Alterations in fatty acid metabolism in response to obesity surgery combined with dietary counseling.

28869586

Obesity

Crucial role of a long-chain fatty acid elongase, Elovl6, in obesity-induced insulin resistance.

17906635

Protein Deficiency

Enzymatic characterization of ELOVL1, a key enzyme in very long-chain fatty acid synthesis.

25499606

Quadriplegia

Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases.

22814679

Retinal Degeneration

ELOVL4 protein preferentially elongates 20:5n3 to very long chain PUFAs over 20:4n6 and 22:6n3.

22158834

Sjogren-Larsson Syndrome

Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases.

22814679

Spinocerebellar Ataxias

Docosahexaenoic acid is a beneficial replacement treatment for spinocerebellar ataxia 38.

28976605

Stargardt Disease

Hetero-oligomeric interactions of an ELOVL4 mutant protein: implications in the molecular mechanism of Stargardt-3 macular dystrophy.

21139992

Starvation

Tissue-specific, nutritional, and developmental regulation of rat fatty acid elongases.

15654130

Tuberculosis

Mycobacterium tuberculosis increases IP-10 and MIG protein despite inhibition of IP-10 and MIG transcription.

21167783

Entries 1 - 20 of 42

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only manually annotated data include textmining data from AMENDAenzyme occurrence in organism, localization and source tissue with reliability ranks include all textmining data from AMENDAenzyme occurrence in organism, localization and source tissue with reliability ranks + FRENDAco-occurrence of enzyme names and organism names (less precise)

ORGANISM

COMMENTARY

LITERATURE

UNIPROT

SEQUENCE DB

SOURCE

Homo sapiens

-

651834, 676089

-

-

brenda

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only manually annotated data include textmining data from AMENDAenzyme occurrence in organism, localization and source tissue with reliability ranks

SOURCE TISSUE

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

SOURCE

liver

Homo sapiens

-

-

651834

brenda

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only manually annotated data include textmining data from AMENDAenzyme occurrence in organism, localization and source tissue with reliability ranks

LOCALIZATION

ORGANISM

UNIPROT

COMMENTARY

GeneOntology No.

LITERATURE

SOURCE

mitochondrion

Homo sapiens

-

-

5739

651834

brenda

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top print hide Go to AA Sequence and Transmembrane Helices SearchGo to Localization Prediction

UNIPROT

ENTRY NAME

ORGANISM

NO. OF AA

NO. OF TRANSM. HELICES

MOLECULAR WEIGHT[Da]

SOURCE

SEQUENCE

LOCALIZATION PREDICTIONThe reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).

P0C7M7 pBLAST

ACSM4_HUMAN

Homo sapiens

580

0

65703

Swiss-Prot

Show Sequence

-

Q08AH1 pBLAST

ACSM1_HUMAN

Homo sapiens

577

0

65273

Swiss-Prot

Show Sequence

-

Q08AH3 pBLAST

ACS2A_HUMAN

Homo sapiens

577

0

64223

Swiss-Prot

Show Sequence

-

Q53FZ2 pBLAST

ACSM3_HUMAN

Homo sapiens

586

0

66153

Swiss-Prot

Show Sequence

-

Q68CK6 pBLAST

ACS2B_HUMAN

Homo sapiens

577

0

64271

Swiss-Prot

Show Sequence

Mitochondrion (Reliability: 1)

Q6NUN0 pBLAST

ACSM5_HUMAN

Homo sapiens

579

0

64760

Swiss-Prot

Show Sequence

-

Q6P461 pBLAST

ACSM6_HUMAN

Homo sapiens

480

0

53585

Swiss-Prot

Show Sequence

-

Q96CM8 pBLAST

ACSF2_HUMAN

Homo sapiens

615

0

68125

Swiss-Prot

Show Sequence

-

A0A087WU25 pBLAST

A0A087WU25_HUMAN

Homo sapiens

200

0

21925

TrEMBL

Show Sequence

Mitochondrion (Reliability: 3)

A8K2Q7 pBLAST

A8K2Q7_HUMAN

Homo sapiens

586

0

66155

TrEMBL

Show Sequence

-

A8K051 pBLAST

A8K051_HUMAN

Homo sapiens

577

0

64260

TrEMBL

Show Sequence

-

B2RAP4 pBLAST

B2RAP4_HUMAN

Homo sapiens

577

0

65257

TrEMBL

Show Sequence

-

B3KP75 pBLAST

B3KP75_HUMAN

Homo sapiens

349

0

38962

TrEMBL

Show Sequence

-

B4DPP3 pBLAST

B4DPP3_HUMAN

Homo sapiens

208

0

23707

TrEMBL

Show Sequence

-

B4DTB9 pBLAST

B4DTB9_HUMAN

Homo sapiens

572

0

63618

TrEMBL

Show Sequence

-

B4DJK7 pBLAST

B4DJK7_HUMAN

Homo sapiens

578

0

65007

TrEMBL

Show Sequence

-

B7Z530 pBLAST

B7Z530_HUMAN

Homo sapiens

498

0

55146

TrEMBL

Show Sequence

-

B7Z8R0 pBLAST

B7Z8R0_HUMAN

Homo sapiens

429

0

47103

TrEMBL

Show Sequence

Secretory Pathway (Reliability: 2)

E5RFK0 pBLAST

E5RFK0_HUMAN

Homo sapiens

154

0

18114

TrEMBL

Show Sequence

other Location (Reliability: 2)

E7EPS5 pBLAST

E7EPS5_HUMAN

Homo sapiens

208

0

23723

TrEMBL

Show Sequence

-

E9PF16 pBLAST

E9PF16_HUMAN

Homo sapiens

572

0

63653

TrEMBL

Show Sequence

-

F5GWL3 pBLAST

F5GWL3_HUMAN

Homo sapiens

498

0

55076

TrEMBL

Show Sequence

-

H0YAU5 pBLAST

H0YAU5_HUMAN

Homo sapiens

249

0

28172

TrEMBL

Show Sequence

-

H3BNJ9 pBLAST

H3BNJ9_HUMAN

Homo sapiens

208

0

23783

TrEMBL

Show Sequence

-

H3BP79 pBLAST

H3BP79_HUMAN

Homo sapiens

61

0

6846

TrEMBL

Show Sequence

-

H3BVD5 pBLAST

H3BVD5_HUMAN

Homo sapiens

158

0

18334

TrEMBL

Show Sequence

-

H3BV29 pBLAST

H3BV29_HUMAN

Homo sapiens

235

0

26334

TrEMBL

Show Sequence

-

H3BSM0 pBLAST

H3BSM0_HUMAN

Homo sapiens

582

0

65532

TrEMBL

Show Sequence

other Location (Reliability: 3)

H3BUF2 pBLAST

H3BUF2_HUMAN

Homo sapiens

135

0

15184

TrEMBL

Show Sequence

-

H3BTX9 pBLAST

H3BTX9_HUMAN

Homo sapiens

498

0

55124

TrEMBL

Show Sequence

-

I3L536 pBLAST

I3L536_HUMAN

Homo sapiens

112

0

12465

TrEMBL

Show Sequence

-

I3L339 pBLAST

I3L339_HUMAN

Homo sapiens

61

0

6800

TrEMBL

Show Sequence

-

V9H1E0 pBLAST

V9H1E0_HUMAN

Homo sapiens

46

0

5065

TrEMBL

Show Sequence

-

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PDB

SCOP

CATH

UNIPROT

ORGANISM

7 entries

Homo sapiens

3B7W

SCOPe (3B7W)

CATH (3B7W)

Q08AH3

Homo sapiens

3GPC

SCOPe (3GPC)

CATH (3GPC)

Q08AH3

Homo sapiens

3C5E

SCOPe (3C5E)

CATH (3C5E)

Q08AH3

Homo sapiens

3DAY

SCOPe (3DAY)

CATH (3DAY)

Q08AH3

Homo sapiens

3EQ6

SCOPe (3EQ6)

CATH (3EQ6)

Q08AH3

Homo sapiens

2WD9

SCOPe (2WD9)

CATH (2WD9)

Q08AH3

Homo sapiens

2VZE

SCOPe (2VZE)

CATH (2VZE)

Q08AH3

Homo sapiens

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PURIFICATION (Commentary)

ORGANISM

UNIPROT

LITERATURE

enzyme form HXM-A and HXM-B

Homo sapiens

-

651834

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CLONED (Commentary)

ORGANISM

UNIPROT

LITERATURE

expression of HXM-A in COS cells

Homo sapiens

-

651834

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APPLICATION

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

medicine

Homo sapiens

-

involvement of the MACS2 Leu513Ser polymorphism in the development of the metabolic syndrome

676089

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REF.

AUTHORS

TITLE

JOURNAL

VOL.

PAGES

YEAR

ORGANISM (UNIPROT)

PUBMED ID

SOURCE

651834

Vessey, D.A.; Lau, E.; Kelley, M.; Warren, R.S.

Isolation, sequencing, and expression of a cDNA for the HXM-A form of xenobiotic/medium-chain fatty acid:CoA ligase from human liver mitochondria

J. Biochem. Mol. Toxicol.

17

1-6

2003

Homo sapiens

12616642

brenda

676089

Lindner, I.; Rubin, D.; Helwig, U.; Nitz, I.; Hampe, J.; Schreiber, S.; Schrezenmeir, J.; Doering, F.

The L513S polymorphism in medium-chain acyl-CoA synthetase 2 (MACS2) is associated with risk factors of the metabolic syndrome in a Caucasian study population

Mol. Nutr. Food Res.

50

270-274

2006

Homo sapiens

16521160

brenda

Entries 1 - 20 of 2

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