BRENDA - Enzyme Database show
show all sequences of 1.1.1.211

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?

Tonin, A.M.; Amaral, A.U.; Busanello, E.N.; Gasparotto, J.; Gelain, D.P.; Gregersen, N.; Wajner, M.; Biochim. Biophys. Acta 1842, 1658-1667 (2014)

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+
-
-
?
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
Rattus norvegicus Wistar
-
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
?
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Rattus norvegicus
Q64428
-
-
Rattus norvegicus Wistar
Q64428
-
-
Source Tissue
Source Tissue
Commentary
Organism
Textmining
brain
-
Rattus norvegicus
-
cerebral cortex
-
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+
-
740102
Rattus norvegicus
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
-
?
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
740102
Rattus norvegicus Wistar
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
-
?
Temperature Optimum [C]
Temperature Optimum [C]
Temperature Optimum Maximum [C]
Commentary
Organism
37
-
assay at
Rattus norvegicus
pH Optimum
pH Optimum Minimum
pH Optimum Maximum
Commentary
Organism
7.2
-
assay at
Rattus norvegicus
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+
-
-
?
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
Rattus norvegicus Wistar
-
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
?
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
brain
-
Rattus norvegicus
-
cerebral cortex
-
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+
-
740102
Rattus norvegicus
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
-
?
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
-
740102
Rattus norvegicus Wistar
a long-chain 3-oxoacyl-CoA + NADH + H+
-
-
-
?
Temperature Optimum [C] (protein specific)
Temperature Optimum [C]
Temperature Optimum Maximum [C]
Commentary
Organism
37
-
assay at
Rattus norvegicus
pH Optimum (protein specific)
pH Optimum Minimum
pH Optimum Maximum
Commentary
Organism
7.2
-
assay at
Rattus norvegicus
Expression
Organism
Commentary
Expression
Rattus norvegicus
nickel strongly represses mitochondrial fatty acid oxidation, the pathway by which fatty acids are catabolized for energy, in both primary human lung fibroblasts and mouse embryonic fibroblasts
down
General Information
General Information
Commentary
Organism
malfunction
Long-chain 3-hydroxylated fatty acids (LCHFA) accumulate in long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein (MTP) deficiencies. 3-Hydroxytetradecanoic acid (3 HTA) reduces mitochondrial membrane potential, NAD(P)H levels, Ca2+ retention capacity and ATP content, besides inducing swelling, cytochrome c release and H2O2 production in Ca2+-loaded mitochondrial preparations. Cyclosporine A plus ADP, as well as ruthenium red, a Ca2+ uptake blocker, prevent these effects, suggesting the involvement of the mitochondrial permeability transition pore (mPTP) and an important role for Ca2+, respectively. 3-Hydroxydodecanoic and 3-hydroxypalmitic acids, that also accumulate in LCHAD and MTP deficiencies, similarly induce mitochondrial swelling and decrease ATP content, but to a variable degree pending on the size of their carbon chain. Pathological neurological phenotype, detailed overview
Rattus norvegicus
physiological function
long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) is part of the mitochondrial trifunctional protein (MTP) complex that also comprises other two enzyme activities, long-chain enoyl-CoA hydratase and long-chain ketoacyl-CoA thiolase (LCKT). This complex is responsible for mitochondrial oxidation of long-chain fatty acids (LCFA)
Rattus norvegicus
General Information (protein specific)
General Information
Commentary
Organism
malfunction
Long-chain 3-hydroxylated fatty acids (LCHFA) accumulate in long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein (MTP) deficiencies. 3-Hydroxytetradecanoic acid (3 HTA) reduces mitochondrial membrane potential, NAD(P)H levels, Ca2+ retention capacity and ATP content, besides inducing swelling, cytochrome c release and H2O2 production in Ca2+-loaded mitochondrial preparations. Cyclosporine A plus ADP, as well as ruthenium red, a Ca2+ uptake blocker, prevent these effects, suggesting the involvement of the mitochondrial permeability transition pore (mPTP) and an important role for Ca2+, respectively. 3-Hydroxydodecanoic and 3-hydroxypalmitic acids, that also accumulate in LCHAD and MTP deficiencies, similarly induce mitochondrial swelling and decrease ATP content, but to a variable degree pending on the size of their carbon chain. Pathological neurological phenotype, detailed overview
Rattus norvegicus
physiological function
long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) is part of the mitochondrial trifunctional protein (MTP) complex that also comprises other two enzyme activities, long-chain enoyl-CoA hydratase and long-chain ketoacyl-CoA thiolase (LCKT). This complex is responsible for mitochondrial oxidation of long-chain fatty acids (LCFA)
Rattus norvegicus
Expression (protein specific)
Organism
Commentary
Expression
Rattus norvegicus
nickel strongly represses mitochondrial fatty acid oxidation, the pathway by which fatty acids are catabolized for energy, in both primary human lung fibroblasts and mouse embryonic fibroblasts
down
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
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1
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2
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1
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1
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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
1-9
2016
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-
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2
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7
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2
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2
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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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-
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2
-
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2
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7
-
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3
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2
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1
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1
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2
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3
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2
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-
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-
1
1
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-
-
740331
Malvagia
Heptadecanoylcarnitine (C17) a ...
Homo sapiens
Clin. Chim. Acta
450
342-348
2015
-
1
-
-
-
-
-
-
-
-
-
-
-
2
-
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-
-
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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
-
-
-
-
-
-
-
-
2
-
-
1
-
3
-
-
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-
-
3
-
-
1
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-
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-
1
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1
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-
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2
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-
1
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3
-
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1
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-
-
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-
-
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-
-
1
1
-
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-
740557
Polinati
Patient-specific induced pluri ...
Homo sapiens
Invest. Ophthalmol. Vis. Sci.
56
3371-3382
2015
-
-
-
-
1
-
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1
-
-
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2
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1
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1
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1
1
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740832
Haglind
Increased and early lipolysis ...
Homo sapiens
J. Inherit. Metab. Dis.
38
315-322
2015
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-
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2
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1
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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13
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1
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1
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-
740102
Tonin
Mitochondrial bioenergetics de ...
Rattus norvegicus, Rattus norvegicus Wistar
Biochim. Biophys. Acta
1842
1658-1667
2014
-
-
-
-
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2
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2
-
7
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3
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2
-
1
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1
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1
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1
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2
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2
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3
-
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2
-
1
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1
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1
2
2
1
-
-
740641
Tonin
Long-chain 3-hydroxy fatty aci ...
Rattus norvegicus, Rattus norvegicus Wistar
J. Bioenerg. Biomembr.
45
47-57
2013
-
-
-
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1
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2
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6
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1
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2
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1
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1
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2
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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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1
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2
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1
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1
1
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723341
Griffin
Mutations in long-chain 3-hydr ...
Homo sapiens
Pediatr. Dev. Pathol.
15
368-374
2012
-
-
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2
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1
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2
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1
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1
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1
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1
1
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696790
Ma
Production of two monomer stru ...
Pseudomonas putida, Pseudomonas putida KT 2240
Biores. Technol.
100
4891-4894
2009
-
1
1
-
1
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2
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710865
Park
Two novel HADHB gene mutations ...
Homo sapiens
Ann. Clin. Lab. Sci.
39
399-404
2009
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1
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1
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1
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686162
Law
A novel functional assay for s ...
Homo sapiens
Clin. Chim. Acta
382
25-30
2007
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1
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1
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688119
Kong
No mutation was found in the a ...
Homo sapiens
J. Gastroenterol. Hepatol.
22
2107-2111
2007
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1
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1
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1
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1
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2
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1
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1
1
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1
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1
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1
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1
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1
1
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-
670281
Gillingham
Effect of optimal dietary ther ...
Homo sapiens
Mol. Genet. Metab.
86
124-133
2005
-
-
-
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1
-
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1
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2
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1
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1
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670476
Oey
Long-chain fatty acid oxidatio ...
Homo sapiens
Pediatr. Res.
57
755-759
2005
-
-
-
-
-
-
-
-
1
-
-
1
-
3
-
-
-
-
-
11
-
-
1
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-
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1
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1
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11
-
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1
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-
-
-
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-
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-
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-
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-
-
-
-
656815
Jones
Effects of odd-numbered medium ...
Homo sapiens
Mol. Genet. Metab.
81
96-99
2004
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1
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2
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1
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1
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1
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656959
Rakheja
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