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Information on EC 5.1.99.6 - NAD(P)H-hydrate epimerase
for references in articles please use BRENDA:EC5.1.99.6
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EC Tree 5 Isomerases
5.1 Racemases and epimerases
5.1.99 Acting on other compounds
5.1.99.6 NAD(P)H-hydrate epimerase
IUBMB Comments
The enzyme can use either (R)-NADH-hydrate or (R)-NADPH-hydrate as a substrate. Its physiological role is to convert the (R) forms to the (S) forms, which could then be restored to active dinucleotides by EC 4.2.1.93, ATP-dependent NAD(P)H-hydrate dehydratase.
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
- 5.1.99.6
-
nadphx
- dehydratase
-
epimerization
-
neurometabolic
- probands
-
moonlighting
-
coma
-
febrile
-
exome
-
edema
-
cerebrospinal
-
ataxia
- pyridoxal
- leukoencephalopathy
- medicine
Reaction Schemes
show=
=
Synonyms
apoa1bp, nad(p)hx epimerase, ynl200c, nad(p)h-hydrate epimerase, more
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
AIBP
epimerase-PPOX
NAD(P)HX epimerase
NAXE
NAD(P)HX epimerase
-
-
-
-
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide = (6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(1)
-
-
-
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate = (6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(2)
-
-
-
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PATHWAY SOURCE
PATHWAYS
MetaCyc
NADH repair (eukaryotes), NADH repair (prokaryotes), NADPH repair (eukaryotes), NADPH repair (prokaryotes)
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SYSTEMATIC NAME
IUBMB Comments
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide 6-epimerase
The enzyme can use either (R)-NADH-hydrate or (R)-NADPH-hydrate as a substrate. Its physiological role is to convert the (R) forms to the (S) forms, which could then be restored to active dinucleotides by EC 4.2.1.93, ATP-dependent NAD(P)H-hydrate dehydratase.
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: -
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: -
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
-
Substrates: i.e. NADHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
-
Substrates: i.e. NADPHX
Products: -
Products: -
r
(R)-NADPH-hydrate
(S)-NADPH-hydrate
-
Substrates: higher catalytic efficiency with (R)-NADPH-hydrate compared to(R)-NADH-hydrate
Products: -
Products: -
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
LITERATURE
COMMENTARY
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: -
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: -
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
Substrates: i.e. NADHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
-
Substrates: i.e. NADHX
Products: -
Products: -
r
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
(6R)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
Substrates: i.e. NADPHX
Products: -
Products: -
r
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
the hydratase is dependent on ATP, but the epimerase ist not
-
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
-
NaCl does not affect activity up to a concentration of 5 mM
-
additional information
-
NaCl does not affect activity up to a concentration of 5 mM
-
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Atherosclerosis
The APOA1bp-SREBF-NOTCH axis is associated with reduced atherosclerosis risk in morbidly obese patients.
Brain Diseases
A novel homozygous missense variant in the NAXE gene in an Iranian family with progressive encephalopathy with brain edema and leukoencephalopathy.
Brain Edema
A novel homozygous missense variant in the NAXE gene in an Iranian family with progressive encephalopathy with brain edema and leukoencephalopathy.
Carcinoma, Hepatocellular
NAD(P)HX epimerase downregulation promotes tumor progression through ROS/HIF-1? signaling in hepatocellular carcinoma.
Dehydration
Homozygous mutation in the APOA1BP is associated with a lethal infantile leukoencephalopathy.
Leukoencephalopathies
A novel homozygous missense variant in the NAXE gene in an Iranian family with progressive encephalopathy with brain edema and leukoencephalopathy.
Leukoencephalopathies
Homozygous mutation in the APOA1BP is associated with a lethal infantile leukoencephalopathy.
Neoplasms
NAD(P)HX epimerase downregulation promotes tumor progression through ROS/HIF-1? signaling in hepatocellular carcinoma.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.0006
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
pH and temperature not specified in the publication
0.00115
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
pH and temperature not specified in the publication
0.0016
(R)-NADH-hydrate
-
in the presence of 5 mM KCl, at 30°C in 25 mM Tris-HCl, pH 8.0, 2 mM MgCl2, 0.2 mM ATP
0.00033
(R)-NADPH-hydrate
-
in the presence of 5 mM KCl, at 30°C in 25 mM Tris-HCl, pH 8.0, 2 mM MgCl2, 0.2 mM ATP
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TURNOVER NUMBER [1/s]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.38
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide
pH and temperature not specified in the publication
0.26
(6S)-6beta-hydroxy-1,4,5,6-tetrahydronicotinamide-adenine dinucleotide phosphate
pH and temperature not specified in the publication
82
(R)-NADH-hydrate
-
in the presence of 5 mM KCl, at 30°C in 25 mM Tris-HCl, pH 8.0, 2 mM MgCl2, 0.2 mM ATP
1827
(R)-NADPH-hydrate
-
in the presence of 5 mM KCl, at 30°C in 25 mM Tris-HCl, pH 8.0, 2 mM MgCl2, 0.2 mM ATP
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
7.1
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
bifunctional NAD(P)H-hydrate repair enzyme Nnr, catalyzes both reactions of EC 5.1.99.6 and 4.2.1.136
UniProt
brenda
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
NAXE is significantly downregulated in hepatocellular carcinoma (HCC) tissues and cell lines
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
Highest Expressing Human Cell Lines
Cell Line LinksPlease wait a moment until the data is sorted. This message will disappear when the data is sorted.
GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
metabolism
the central cofactors NAD(P)H are prone to damage by hydration, resulting in formation of redox-inactive derivatives designated NAD(P)HX. The highly conserved enzymes NAD(P)HX dehydratase (NAXD) and NAD(P)HX epimerase (NAXE) function to repair intracellular NAD(P)HX. The ATP-dependent metabolite repair enzyme NAD(P)HX dehydratase (NAXD) acts specifically on the S epimer of NAD(P)HX. The second enzyme, NAD(P)HX epimerase (NAXE), catalyses the interconversion between the S- and R-forms of NAD(P)HX, and essentially supplies the substrate for the stereospecific enzyme NAXD. NAD(P)H damage and NAD(P)HX repair by NAXD and NAXE, overview
physiological function
additional information
malfunction
a truncated form of the Arabidopsis epimerase lacking the PPOX domain is also fully active in the interconversion
malfunction
NAD(P)HX epimerase downregulation promotes tumor progression through ROS/HIF-1alpha signaling in hepatocellular carcinoma
malfunction
pathogenic variants in both the NAXD and NAXE genes are associated with rapid deterioration and death after an otherwise trivial fever, infection, or illness in young patients. As more patients are identified, distinct clinical features are emerging depending on the location of the pathogenic variant. The clinical features of all published NAXD deficiency patients are analyzed and distinct patterns in clinical presentations are found depending on which subcellular compartment is affected by the enzymatic deficiency, overview. Cytosolic NAD(P)HX repair may protect from neurological damage, whereas muscle fibres may be more sensitive to mitochondrial NAD(P)HX damage. Children with NAXE deficiency have an almost identical, nearly always fatal neurodegenerative course. NAXD and NAXE are critical metabolite repair enzymes, and physical or heat stress can damage the central metabolite NAD(P)H to form a toxic compound NAD(P)HX, and it is likely that the fever or illness-induced stress in children triggers a rapid accumulation of NAD(P)HX which cannot be effectively cleared due to a lack of NAXD (or NAXE) enzymes
physiological function
hydration of NAD(P)H to NAD(P)HX, which inhibits several dehydrogenases, is corrected by an ATP-dependent dehydratase and an epimerase recently identified as the products of the vertebrate Carkd (carbohydrate kinase domain) and Aibp (apolipoprotein AI-binding protein) genes, respectively
physiological function
hydration of NAD(P)H to NAD(P)HX, which inhibits several dehydrogenases, is corrected by an ATP-dependent dehydratase and an epimerase recently identified as the products of the vertebrate Carkd (carbohydrate kinase domain) and Aibp (apolipoprotein AI-binding protein) genes, respectively
physiological function
hydration of NAD(P)H to NAD(P)HX, which inhibits several dehydrogenases, is corrected by an ATP-dependent dehydratase and an epimerase recently identified as the products of the vertebrate Carkd (carbohydrate kinase domain) and Aibp (apolipoprotein AI-binding protein) genes, respectively
physiological function
NADH and NADPH undergo spontaneous and enzymatic reactions that produce R and S forms of NAD(P)H hydrates, which are not electron donors and inhibit various dehydrogenases. The highly conserved epimerase repairs the hydrates formed from NADH and NADPH in plants together with a highly conserved dehydratase targeted to multiple compartments
physiological function
-
NADH and NADPH undergo spontaneous and enzymatic reactions that produce R and S forms of NAD(P)H hydrates, which are not electron donors and inhibit various dehydrogenases. The highly conserved epimerase repairs the hydrates formed from NADH and NADPH in plants together with a highly conserved dehydratase targeted to multiple compartments
physiological function
enzyme NAD(P)HX epimerase (NAXE) is an epimerase that allows the repair of damaged forms of antioxidant NADPH, it is a potential cellular ROS scavenger. A tumor suppressor role is found for NAXE in HCC by scavenging excessive ROS and inhibiting tumor-promoting signaling pathways, suggesting a strategy for HCC therapy by targeting redox signaling. NAXE inhibits HIF-1alpha signaling by promoting proteasomal degradation in HCC (by eliminating ROS in HCC). Activated HIF-1alpha signaling is associated with NAXE downregulation-induced HCC migration, invasion, and EMT
physiological function
the central cofactors NAD(P)H are prone to damage by hydration, resulting in formation of redox-inactive derivatives designated NAD(P)HX. The highly conserved enzyme NAD(P)HX epimerase (NAXE) functions to repair intracellular NAD(P)HX. NAD(P)HX epimerase (NAXE) catalyses the interconversion between the S- and R-forms of NAD(P)HX, and essentially supplies the substrate for the stereospecific enzyme NAXD
additional information
the epimerase homologue is fused to the vitamin B6 salvage enzyme pyridoxine 5'-phosphate oxidase, PPOX. Plant epimerase homologs have an extra C-terminal domain that belongs to the pyridoxine/pyridoxamine phosphate oxidase (PPOX) family
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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
K192A
mutation decreases in vitro epimerase activity by more than 95% but does not affect dehydratase activity, reaction of EC 4.2.1.136. Mutant cells have essentially normal NAD(P)HX dehydratase activity and NAD(P)HX levels. Cells show metabolome changes, particularly in amino acids, which exceed those in cells lacking the entire yjeF gene. Mutant cells also have reduced levels of free (i.e. weakly bound or unbound) pyridoxal 5'-phosphate
additional information
knockdown of NAXE expression in HCC cells promotes migration, invasion, and metastasis by inducing epithelial-mesenchymal transition (EMT), whereas NAXE overexpression causes the opposite effects. Mechanistically, low NAXE expression reduces NADPH levels and further causes ROS level increase and hypoxia-inducible factor-1alpha (HIF-1alpha) activation, thereby promoting invasion and metastasis of HCC by facilitating EMT. Knockdown of NAXE expression in HepG2 dramatically downregulates mRNA and protein levels of the epithelial marker E-cadherin, whereas mesenchymal markers, such as vimentin and N-cadherin, are significantly upregulated. In addition, only Twist expression, but not other 2 classical EMT-associated transcription factors, Snail and ZEB, is increased after inhibiting NAXE expression in Hep-G2 cells. In contrast, NAXE overexpression in Huh7 cells induces the opposite results
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PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
Ni2+- or Co2+-charged column chromatography and Q-Sepharose column chromatography
recombinant N-terminally His-tagged enzyme from Escherichia coli by nickel affinity chromatography
Ni2+- or Co2+-charged column chromatography and Q-Sepharose column chromatography
-
Ni2+- or Co2+-charged column chromatography and Q-Sepharose column chromatography
-
recombinant N-terminally His-tagged enzyme from Escherichia coli by nickel affinity chromatography
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CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expressed in Escherichia coli BL21(DE3) cells
expression of N-terminally His-tagged enzyme in Escherichia coli
gene Aibp, expression of GFP-fused or Myc-tagged mAIBP in CHO cells and in HEK-293 cells, in vitro transcription-translation assays, full-length coding sequences of the mouse mAIBP
quantitative RT-PCR enzyme expression analysis in L02 and HCC cell lines, NAXE overexpression in Huh7 cells
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
diagnostics
NAXE downregulation is associated with poor clinicopathological characteristics and is an independent risk factor for overall and disease-free survival of in hepatocellular carcinoma (HCC) patients after liver resection. In addition, low NAXE expression can identify worse prognosis of HCC patients before vascular invasion or in early stages of disease. In particularly, low NAXE expression in HCC is markedly associated with microvascular invasion (MVI) and its combination with MVI predicts poorer prognosis of HCC patients after liver resection
medicine
pathogenic biallelic mutations in NAXE in children from four families lead to (sub-)acute-onset ataxia, cerebellar edema, spinal myelopathy, and skin lesions. Lactate is elevated in cerebrospinal fluid of all affected individuals. Disease onset is during the second year of life and clinical signs as well as episodes of deterioration are triggered by febrile infections. Disease course is rapidly progressive, leading to coma, global brain atrophy, and finally to death in all affected individuals. NAXE levels are undetectable in fibroblasts from affected individuals of two families. These fibroblasts show highly elevated concentrations of the toxic metabolite cyclic-NADHX
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Marbaix, A.; Nol, G.; Detroux, A.; Vertommen, D.; Van Schaftingen, E.; Linster, C.
Extremely conserved ATP- or ADP-dependent enzymatic system for nicotinamide nucleotide
J. Biol. Chem.
286
41246-41252
2011
Saccharomyces cerevisiae, Mus musculus
brenda
Marbaix, A.Y.; Tyteca, D.; Niehaus, T.D.; Hanson, A.D.; Linster, C.L.; Van Schaftingen, E.
Occurrence and subcellular distribution of the NAD(P)HX repair system in mammals
Biochem. J.
460
49-58
2014
Mus musculus (Q8K4Z3), Rattus norvegicus (B0BNM1), Homo sapiens (Q8NCW5)
brenda
Niehaus, T.D.; Richardson, L.G.; Gidda, S.K.; Elbadawi-Sidhu, M.; Meissen, J.K.; Mullen, R.T.; Fiehn, O.; Hanson, A.D.
Plants utilize a highly conserved system for repair of NADH and NADPH hydrates
Plant Physiol.
165
52-61
2014
Arabidopsis thaliana (Q9LTX3), Zea mays
brenda
Kremer, L.S.; Danhauser, K.; Herebian, D.; Petkovic Ramadza, D.; Piekutowska-Abramczuk, D.; Seibt, A.; Mueller-Felber, W.; Haack, T.B.; Ploski, R.; Lohmeier, K.; Schneider, D.; Klee, D.; Rokicki, D.; Mayatepek, E.; Strom, T.M.; Meitinger, T.; Klopstock, T.; Pronicka, E.; Mayr, J.A.; Baric, I.; Distelmaier, F.; Prokisch, H.
NAXE mutations disrupt the cellular NAD(P)HX repair system and cause a lethal neurometabolic disorder of early childhood
Am. J. Hum. Genet.
99
894-902
2016
Homo sapiens (Q8NCW5), Homo sapiens
brenda
Niehaus, T.D.; Elbadawi-Sidhu, M.; Huang, L.; Prunetti, L.; Gregory, J.F.; de Crecy-Lagard, V.; Fiehn, O.; Hanson, A.D.
Evidence that the metabolite repair enzyme NAD(P)HX epimerase has a moonlighting function
Biosci. Rep.
38
BSR20180223
2018
Escherichia coli (P31806), Escherichia coli
brenda
Sun, B.; Yu, L.; Xu, C.; Li, Y.M.; Zhao, Y.R.; Cao, M.M.; Yang, L.Y.
NAD(P)HX epimerase downregulation promotes tumor progression through ROS/HIF-1alpha signaling in hepatocellular carcinoma
Cancer Sci.
112
2753-2769
2021
Homo sapiens (A0A7P0Z426)
brenda
Van Bergen, N.J.; Walvekar, A.S.; Patraskaki, M.; Sikora, T.; Linster, C.L.; Christodoulou, J.
Clinical and biochemical distinctions for a metabolite repair disorder caused by NAXD or NAXE deficiency
J. Inherit. Metab. Dis.
45
1028-1038
2022
Homo sapiens (A0A7P0Z426)
brenda
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Release 2026.1 (March 2026)
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