HIBADH Antikörper (AA 251-336) (AbBy Fluor® 350)
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- Target Alle HIBADH Antikörper anzeigen
- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
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Bindungsspezifität
- AA 251-336
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Reaktivität
- Human, Maus
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Wirt
- Kaninchen
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Klonalität
- Polyklonal
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Konjugat
- Dieser HIBADH Antikörper ist konjugiert mit AbBy Fluor® 350
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Applikation
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- Kreuzreaktivität
- Human, Maus
- Homologie
- Rat,Cow,Sheep,Horse
- Aufreinigung
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human HIBADH
- Isotyp
- IgG
- Top Product
- Discover our top product HIBADH Primärantikörper
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- Applikationshinweise
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - Beschränkungen
- Nur für Forschungszwecke einsetzbar
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- Format
- Liquid
- Konzentration
- 1 μg/μL
- Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- Konservierungsmittel
- ProClin
- Vorsichtsmaßnahmen
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- Lagerung
- -20 °C
- Informationen zur Lagerung
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Haltbarkeit
- 12 months
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- Target
- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
- Andere Bezeichnung
- Hibadh (HIBADH Produkte)
- Synonyme
- NS5ATP1 antikoerper, 6430402H10Rik antikoerper, AI265272 antikoerper, hibadh antikoerper, zgc:66262 antikoerper, wu:fb07f02 antikoerper, zgc:100804 antikoerper, 3-hydroxyisobutyrate dehydrogenase antikoerper, 3-hydroxyisobutyrate dehydrogenase b antikoerper, 3-hydroxyisobutyrate dehydrogenase S homeolog antikoerper, 3-hydroxyisobutyrate dehydrogenase a antikoerper, HIBADH antikoerper, Hibadh antikoerper, hibadhb antikoerper, hibadh.S antikoerper, hibadha antikoerper
- Hintergrund
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Synonyms: 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase, 3 hydroxyisobutyrate dehydrogenase, 3 hydroxyisobutyrate dehydrogenase mitochondrial, EC 1.1.1.31, MGC40361, NS5ATP1, 3HIDH_HUMAN.
Background: HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization 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 hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized 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.
- Gen-ID
- 11112
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