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show all sequences of 1.1.1.211

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

Cecatto, C.; Hickmann, F.H.; Rodrigues, M.D.; Amaral, A.U.; Wajner, M.; FEBS J. 282, 4714-4726 (2015)

Data extracted from this reference:

Localization
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Rattus norvegicus
5739
-
Natural Substrates/ Products (Substrates)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
Rattus norvegicus
-
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
?
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Rattus norvegicus
Q64428
-
-
Source Tissue
Source Tissue
Commentary
Organism
Textmining
brain
-
Rattus norvegicus
-
heart
-
Rattus norvegicus
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
740438
Rattus norvegicus
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
-
?
Cofactor
Cofactor
Commentary
Organism
Structure
NAD+
-
Rattus norvegicus
Cofactor (protein specific)
Cofactor
Commentary
Organism
Structure
NAD+
-
Rattus norvegicus
Localization (protein specific)
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Rattus norvegicus
5739
-
Natural Substrates/ Products (Substrates) (protein specific)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
Rattus norvegicus
-
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
?
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
brain
-
Rattus norvegicus
-
heart
-
Rattus norvegicus
-
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
740438
Rattus norvegicus
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
-
?
General Information
General Information
Commentary
Organism
malfunction
mitochondrial trifunctional protein and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiencies are fatty acid oxidation disorders biochemically characterized by tissue accumulation of long-chain fatty acids and derivatives, including the monocarboxylic long-chain 3-hydroxy fatty acids (LCHFAs) 3-hydroxytetradecanoic acid (3HTA) and 3-hydroxypalmitic acid (3HPA). Deregulation of mitochondrial functions can be provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart. 3HTA and 3HPA significantly decrease mitochondrial membrane potential, the matrix NAD(P)H pool and Ca2+ retention capacity, and also induced mitochondrial swelling. These fatty acids also provoke a marked decrease of ATP production reflecting severe energy dysfunction. 3HTA-induced mitochondrial alterations are completely prevented by the classical mitochondrial permeability transition (mPT) inhibitors cyclosporin A and ADP, as well as by ruthenium red, a Ca2+ uptake blocker, indicating that LCHFAs induce Ca2+-dependent mPT pore opening. Comparison of the susceptibility of heart and brain to the toxic effects of these hydroxylated fatty acids, phenotypes, overview
Rattus norvegicus
General Information (protein specific)
General Information
Commentary
Organism
malfunction
mitochondrial trifunctional protein and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiencies are fatty acid oxidation disorders biochemically characterized by tissue accumulation of long-chain fatty acids and derivatives, including the monocarboxylic long-chain 3-hydroxy fatty acids (LCHFAs) 3-hydroxytetradecanoic acid (3HTA) and 3-hydroxypalmitic acid (3HPA). Deregulation of mitochondrial functions can be provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart. 3HTA and 3HPA significantly decrease mitochondrial membrane potential, the matrix NAD(P)H pool and Ca2+ retention capacity, and also induced mitochondrial swelling. These fatty acids also provoke a marked decrease of ATP production reflecting severe energy dysfunction. 3HTA-induced mitochondrial alterations are completely prevented by the classical mitochondrial permeability transition (mPT) inhibitors cyclosporin A and ADP, as well as by ruthenium red, a Ca2+ uptake blocker, indicating that LCHFAs induce Ca2+-dependent mPT pore opening. Comparison of the susceptibility of heart and brain to the toxic effects of these hydroxylated fatty acids, phenotypes, overview
Rattus norvegicus
Other publictions for EC 1.1.1.211
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [°C]
Temperature Range [°C]
Temperature Stability [°C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [°C] (protein specific)
Temperature Range [°C] (protein specific)
Temperature Stability [°C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
740890
Erdol
An unusual case of LCHAD defic ...
Homo sapiens
J. Pediatr. Hematol. Oncol.
38
661-662
2016
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1
1
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741457
Cecatto
Disturbance of mitochondrial f ...
Rattus norvegicus, Rattus norvegicus Wistar
Toxicol. In Vitro
36
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2016
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1
1
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740106
Hickmann
Uncoupling, metabolic inhibiti ...
Rattus norvegicus, Rattus norvegicus Wistar
Biochim. Biophys. Acta
1847
620-628
2015
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1
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740331
Malvagia
Heptadecanoylcarnitine (C17) a ...
Homo sapiens
Clin. Chim. Acta
450
342-348
2015
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1
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1
1
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740438
Cecatto
Deregulation of mitochondrial ...
Rattus norvegicus
FEBS J.
282
4714-4726
2015
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2
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1
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Polinati
Patient-specific induced pluri ...
Homo sapiens
Invest. Ophthalmol. Vis. Sci.
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3371-3382
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740832
Haglind
Increased and early lipolysis ...
Homo sapiens
J. Inherit. Metab. Dis.
38
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2015
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1
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741474
Han
Expression of long chain fatty ...
Mus musculus
Zhonghua Yi Xue Za Zhi
95
26-29
2015
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2
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13
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1
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Tonin
Mitochondrial bioenergetics de ...
Rattus norvegicus, Rattus norvegicus Wistar
Biochim. Biophys. Acta
1842
1658-1667
2014
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1
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Tonin
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2
2
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741064
Anderson
When the usual symptoms become ...
Homo sapiens
Neonatal Netw.
32
262-273
2013
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Griffin
Mutations in long-chain 3-hydr ...
Homo sapiens
Pediatr. Dev. Pathol.
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368-374
2012
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2
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1
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Pseudomonas putida, Pseudomonas putida KT 2240
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100
4891-4894
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Two novel HADHB gene mutations ...
Homo sapiens
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2009
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A novel functional assay for s ...
Homo sapiens
Clin. Chim. Acta
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Homo sapiens
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Gillingham
Effect of optimal dietary ther ...
Homo sapiens
Mol. Genet. Metab.
86
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Long-chain fatty acid oxidatio ...
Homo sapiens
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11
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1
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11
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656815
Jones
Effects of odd-numbered medium ...
Homo sapiens
Mol. Genet. Metab.
81
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1
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656959
Rakheja
Evidence for fatty acid oxidat ...
Homo sapiens
Placenta
23
447-450
2002
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288673
Middleton
The mitochondrial long-chain t ...
Homo sapiens
Biochem. Soc. Trans.
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427-431
1994
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286270
Carpenter
Human liver long-chain 3-hydro ...
Homo sapiens
Biochem. Biophys. Res. Commun.
183
443 - 448
1992
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5
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1
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