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Artikel nummer : (BOSSBS-15483R-CY3)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Artikel nummer : (1288339.)
Leverantör: USP
Beskrivning: USP Reference Standards are specified for use in conducting official USP–NF tests and assays. USP also provides Reference Standards specified in the Food Chemicals Codex as well as authentic substances—high-quality chemical samples—as a service to analytical, clinical, pharmaceutical and research laboratories. To confirm accuracy and reproducibility, USP Reference Standards are rigorously tested and evaluated by multiple independent laboratories including USP, commercial, regulatory, and academic labs. USP also provide publicly available, official documentary standards for pharmaceutical ingredients in the USP–NF that link directly with our primary reference standards.
UOM: 1 * 10 mg


Artikel nummer : (BOSSBS-2698R-A750)
Leverantör: Bioss
Beskrivning: Proton-linked monocarboxylate transporter. catalyses the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate (By similarity).
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-15483R-A555)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-2698R-HRP)
Leverantör: Bioss
Beskrivning: Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate (By similarity).
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-15483R-A647)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Artikel nummer : (MOLEM35191540)
Leverantör: Molekula
Beskrivning: D-(-)-Valine
UOM: 1 * 5 g

Market Source Item This is a MarketSource item. Additional charges may apply

Leverantör: TCI
Beskrivning: L-(-)-tert-Leucine (H-Tle-OH) ≥98.0%

Artikel nummer : (BOSSBS-15483R-FITC)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-15483R-HRP)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Leverantör: MP Biomedicals
Beskrivning: D-(-)-Valine ≥99%, pulver

Artikel nummer : (MOLEM51907913)
Leverantör: Molekula
Beskrivning: N-Boc-L-valine
UOM: 1 * 25 g

Market Source Item This is a MarketSource item. Additional charges may apply

Artikel nummer : (04-12-0029-25)
Leverantör: Novabiochem (Part of Merck)
Beskrivning: N-Boc-L-valine
UOM: 1 * 25 g


Artikel nummer : (BOSSBS-15483R-A350)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Artikel nummer : (BOSSBS-15483R-A680)
Leverantör: Bioss
Beskrivning: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Leverantör: Molekula
Beskrivning: L (+)-Valin

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Lager för denna produkt är begränsat, men kan vara tillgänglig i ett lager nära dig. Se till att du är inloggad på hemsidan så att tillgängligt lager kan visas. Om call fortfarande visas och du behöver hjälp, ring oss på 08-621 34 20.
VWR International AB säljer bara etanol till skattebefriade kunder. Denna produkt ä reglerad enligt svensk lagstiftning. Om ytterligare information krävs av dig kommer du att kontaktas via e-post.
VWR International AB säljer bara etanol till skattebefriade kunder.
Denna produkt ä reglerad enligt svensk lagstiftning. Om ytterligare information krävs av dig kommer du att kontaktas via email.
Produkten har blockerats av din organisation. Vänligen kontakta din inköpsavdelning för mer information.
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Denna produkt är inte längre tillgänglig. Alternativ kan vara tillgängliga genom att söka med VWR art.nr ovan. Om du behöver ytterligare hjälp, ring VWR Kundservice på 08-621 34 20.
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