HIBADH anticorps (AA 251-336) (AbBy Fluor® 488)
-
- Antigène Voir toutes HIBADH Anticorps
- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
-
Épitope
- AA 251-336
-
Reactivité
- Humain, Souris
-
Hôte
- Lapin
-
Clonalité
- Polyclonal
-
Conjugué
- Cet anticorp HIBADH est conjugé à/à la AbBy Fluor® 488
-
Application
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- Réactivité croisée
- Humain, Souris
- Homologie
- Rat,Cow,Sheep,Horse
- Purification
- Purified by Protein A.
- Immunogène
- KLH conjugated synthetic peptide derived from human HIBADH
- Isotype
- IgG
- Top Product
- Discover our top product HIBADH Anticorps primaire
-
-
- Indications d'application
-
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - Restrictions
- For Research Use only
-
- Format
- Liquid
- Concentration
- 1 μg/μL
- Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- Agent conservateur
- ProClin
- Précaution d'utilisation
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- Stock
- -20 °C
- Stockage commentaire
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Date de péremption
- 12 months
-
- Antigène
- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
- Autre désignation
- Hibadh (HIBADH Produits)
- Synonymes
- anticorps NS5ATP1, anticorps 6430402H10Rik, anticorps AI265272, anticorps hibadh, anticorps zgc:66262, anticorps wu:fb07f02, anticorps zgc:100804, anticorps 3-hydroxyisobutyrate dehydrogenase, anticorps 3-hydroxyisobutyrate dehydrogenase b, anticorps 3-hydroxyisobutyrate dehydrogenase S homeolog, anticorps 3-hydroxyisobutyrate dehydrogenase a, anticorps HIBADH, anticorps Hibadh, anticorps hibadhb, anticorps hibadh.S, anticorps hibadha
- Sujet
-
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.
- ID gène
- 11112
-