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1.1.1.211: long-chain-3-hydroxyacyl-CoA dehydrogenase

This is an abbreviated version!
For detailed information about long-chain-3-hydroxyacyl-CoA dehydrogenase, go to the full flat file.

Word Map on EC 1.1.1.211

Reaction

a long-chain (S)-3-hydroxyacyl-CoA
+
NAD+
=
a long-chain 3-oxoacyl-CoA
+
NADH
+
H+

Synonyms

2-enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase/3-oxoacyl-CoA thiolase, 3-hydroxyacyl-CoA dehydrogenase, beta-hydroxyacyl-CoA dehydrogenase, enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein, HADHA, LCHAD, LHCAD, long chain 3-hydroxyacyl-CoA dehydrogenase, long chain fatty acid oxidase, long-chain 3-hydroxy acyl-CoA dehydrogenase, long-chain 3-hydroxy-acyl-CoA dehydrogenase, long-chain 3-hydroxyacyl coenzyme A dehydrogenase, long-chain 3-hydroxyacyl-CoA dehydrogenase, long-chain L-3 hydroxyacyl-CoA dehydrogenase, mitochondrial trifunctional protein, More, MTP

ECTree

     1 Oxidoreductases
         1.1 Acting on the CH-OH group of donors
             1.1.1 With NAD+ or NADP+ as acceptor
                1.1.1.211 long-chain-3-hydroxyacyl-CoA dehydrogenase

Reference

Reference on EC 1.1.1.211 - long-chain-3-hydroxyacyl-CoA dehydrogenase

Please use the Reference Search for a specific query.
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
El-Fakhri, M.; Middleton, B.
The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria
Biochim. Biophys. Acta
713
270-279
1982
Oryctolagus cuniculus, Rattus norvegicus
Manually annotated by BRENDA team
Carpenter, K.; Pollitt, R.J.; Middleton, B.
Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria
Biochem. Biophys. Res. Commun.
183
443 - 448
1992
Homo sapiens
Manually annotated by BRENDA team
Middleton, B.
The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA thiolase
Biochem. Soc. Trans.
22
427-431
1994
Homo sapiens
Manually annotated by BRENDA team
Uchida, Y.; Izai, K.; Orii, T.; Hashimoto, T.
Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. II. Purification and properties of enoyl-coenzyme A (CoA) hydratase/3-hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase trifunctional protein
J. Biol. Chem.
267
1034-1041
1992
Rattus norvegicus (Q64428)
Manually annotated by BRENDA team
Carpenter, K.; Pollitt, R.J.; Middleton, B.
A unique, membrane-bound, multifunctional enzyme from human liver mitochondria catalysing three steps of fatty acid beta-oxidation
Biochem. Soc. Trans.
21
35S
1992
Homo sapiens
Manually annotated by BRENDA team
Jones, P.M.; Butt, Y.M.; Bennett, M.J.
Effects of odd-numbered medium-chain fatty acids on the accumulation of long-chain 3-hydroxy-fatty acids in long-chain L-3-hydroxyacyl CoA dehydrogenase and mitochondrial trifunctional protein deficient skin fibroblasts
Mol. Genet. Metab.
81
96-99
2004
Homo sapiens
Manually annotated by BRENDA team
Rakheja, D.; Bennett, M.J.; Foster, B.M.; Domiati-Saad, R.; Rogers, B.B.
Evidence for fatty acid oxidation in human placenta, and the relationship of fatty acid oxidation enzyme activities with gestational age
Placenta
23
447-450
2002
Homo sapiens
Manually annotated by BRENDA team
Gillingham, M.B.; Weleber, R.G.; Neuringer, M.; Connor, W.E.; Mills, M.; van Calcar, S.; Ver Hoeve, J.; Wolff, J.; Harding, C.O.
Effect of optimal dietary therapy upon visual function in children with long-chain 3-hydroxyacyl CoA dehydrogenase and trifunctional protein deficiency
Mol. Genet. Metab.
86
124-133
2005
Homo sapiens
Manually annotated by BRENDA team
Oey, N.A.; den Boer, M.E.; Wijburg, F.A.; Vekemans, M.; Auge, J.; Steiner, C.; Wanders, R.J.; Waterham, H.R.; Ruiter, J.P.; Attie-Bitach, T.
Long-chain fatty acid oxidation during early human development
Pediatr. Res.
57
755-759
2005
Homo sapiens
Manually annotated by BRENDA team
Law, L.K.; Tang, N.L.; Hui, J.; Ho, C.S.; Ruiter, J.; Fok, T.F.; Wanders, R.J.; Lam, C.W.
A novel functional assay for simultaneous determination of total fatty acid beta-oxidation flux and acylcarnitine profiling in human skin fibroblasts using (2)H(31)-palmitate by isotope ratio mass spectrometry and electrospray tandem mass spectrometry
Clin. Chim. Acta
382
25-30
2007
Homo sapiens
Manually annotated by BRENDA team
Kong, X.F.; Zhang, X.X.; Yu, Y.Y.; Shi, Q.; La, D.D.; Zhu-Ge, C.D.; Deng, L.; Gong, Q.M.; Shen, B.Y.; Peng, C.H.; Li, H.W.
No mutation was found in the alpha-subunit of the mitochondrial tri-functional protein in one patient with severe acute fatty liver of pregnancy and her relatives
J. Gastroenterol. Hepatol.
22
2107-2111
2007
Homo sapiens
Manually annotated by BRENDA team
Ma, L.; Zhang, H.; Liu, Q.; Chen, J.; Zhang, J.; Chen, G.Q.
Production of two monomer structures containing medium-chain-length polyhydroxyalkanoates by beta-oxidation-impaired mutant of Pseudomonas putida KT2442
Biores. Technol.
100
4891-4894
2009
Pseudomonas putida (Q88L88), Pseudomonas putida KT 2240 (Q88L88)
Manually annotated by BRENDA team
Park, H.D.; Kim, S.R.; Ki, C.S.; Lee, S.Y.; Chang, Y.S.; Jin, D.K.; Park, W.S.
Two novel HADHB gene mutations in a Korean patient with mitochondrial trifunctional protein deficiency
Ann. Clin. Lab. Sci.
39
399-404
2009
Homo sapiens
Manually annotated by BRENDA team
Griffin, A.C.; Strauss, A.W.; Bennett, M.J.; Ernst, L.M.
Mutations in long-chain 3-hydroxyacyl coenzyme A dehydrogenase are associated with placental maternal floor infarction/massive perivillous fibrin deposition
Pediatr. Dev. Pathol.
15
368-374
2012
Homo sapiens
Manually annotated by BRENDA team
Tonin, A.M.; Amaral, A.U.; Busanello, E.N.; Gasparotto, J.; Gelain, D.P.; Gregersen, N.; Wajner, M.
Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: a possible role of mPTP opening as a pathomechanism in these disorders?
Biochim. Biophys. Acta
1842
1658-1667
2014
Rattus norvegicus (Q64428), Rattus norvegicus Wistar (Q64428)
Manually annotated by BRENDA team
Hickmann, F.H.; Cecatto, C.; Kleemann, D.; Monteiro, W.O.; Castilho, R.F.; Amaral, A.U.; Wajner, M.
Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency
Biochim. Biophys. Acta
1847
620-628
2015
Rattus norvegicus (Q64428), Rattus norvegicus Wistar (Q64428)
Manually annotated by BRENDA team
Malvagia, S.; Haynes, C.A.; Grisotto, L.; Ombrone, D.; Funghini, S.; Moretti, E.; McGreevy, K.S.; Biggeri, A.; Guerrini, R.; Yahyaoui, R.; Garg, U.; Seeterlin, M.; Chace, D.; De Jesus, V.R.; la Marca, G.
Heptadecanoylcarnitine (C17) a novel candidate biomarker for newborn screening of propionic and methylmalonic acidemias
Clin. Chim. Acta
450
342-348
2015
Homo sapiens (P40939), Homo sapiens
Manually annotated by BRENDA team
Cecatto, C.; Hickmann, F.H.; Rodrigues, M.D.; Amaral, A.U.; Wajner, M.
Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart - mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders
FEBS J.
282
4714-4726
2015
Rattus norvegicus (Q64428)
Manually annotated by BRENDA team
Polinati, P.P.; Ilmarinen, T.; Trokovic, R.; Hyotylainen, T.; Otonkoski, T.; Suomalainen, A.; Skottman, H.; Tyni, T.
Patient-specific induced pluripotent stem cell-derived RPE cells: understanding the pathogenesis of retinopathy in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
Invest. Ophthalmol. Vis. Sci.
56
3371-3382
2015
Homo sapiens (P40939), Homo sapiens
Manually annotated by BRENDA team
Tonin, A.; Amaral, A.; Busanello, E.; Grings, M.; Castilho, R.; Wajner, M.
Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria
J. Bioenerg. Biomembr.
45
47-57
2013
Rattus norvegicus (Q64428), Rattus norvegicus Wistar (Q64428)
Manually annotated by BRENDA team
Haglind, C.; Nordenstroem, A.; Ask, S.; von Doebeln, U.; Gustafsson, J.; Stenlid, M.
Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast
J. Inherit. Metab. Dis.
38
315-322
2015
Homo sapiens (P40939), Homo sapiens
Manually annotated by BRENDA team
Erdol, S.; Ture, M.; Baytan, B.; Yakut, T.; Saglam, H.
An unusual case of LCHAD deficiency presenting with a clinical picture of hemophagocytic lymphohistiocytosis: secondary HLH or coincidence?
J. Pediatr. Hematol. Oncol.
38
661-662
2016
Homo sapiens (P40939), Homo sapiens
Manually annotated by BRENDA team
Anderson, S.; Brooks, S.S.
When the usual symptoms become an unusual diagnosis: a case report of trifunctional protein complex
Neonatal Netw.
32
262-273
2013
Homo sapiens (P40939), Homo sapiens
Manually annotated by BRENDA team
Cecatto, C.; Godoy, K.d.o.s..S.; da Silva, J.C.; Amaral, A.U.; Wajner, M.
Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle
Toxicol. In Vitro
36
1-9
2016
Rattus norvegicus (Q64428), Rattus norvegicus Wistar (Q64428)
Manually annotated by BRENDA team
Han, Y.; Yang, Z.; Ding, X.; Yu, H.; Yi, Y.
Expression of long chain fatty acid oxidase in maternal and fetal tissues in preeclampsia-like mouse model in mid-gestation
Zhonghua Yi Xue Za Zhi
95
26-29
2015
Mus musculus
Manually annotated by BRENDA team
Stinton, C.; Fraser, H.; Geppert, J.; Johnson, R.; Connock, M.; Johnson, S.; Clarke, A.; Taylor-Phillips, S.
Newborn screening for long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies using acylcarnitines measurement in dried blood spots - a systematic review of test accuracy
Front. Pediatr.
9
606194
2021
Homo sapiens
Manually annotated by BRENDA team
Khare, T.; Khare, S.; Angdisen, J.J.; Zhang, Q.; Stuckel, A.; Mooney, B.P.; Ridenhour, S.E.; Gitan, R.S.; Hammoud, G.M.; Ibdah, J.A.
Defects in long-chain 3-hydroxy acyl-CoA dehydrogenase lead to hepatocellular carcinoma A novel etiology of hepatocellular carcinoma
Int. J. Cancer
147
1461-1473
2020
Mus musculus, Homo sapiens (P40939), Homo sapiens
Manually annotated by BRENDA team