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Anti-HIBADH Polyclonal Antibody
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Catalog No. TMAB-07020
Anti-HIBADH Polyclonal Antibody is a Rabbit antibody targeting HIBADH. Anti-HIBADH Polyclonal Antibody can be used in WB.
Anti-HIBADH Polyclonal Antibody
Anti-HIBADH Polyclonal Antibody
😃Good
Catalog No. TMAB-07020
Anti-HIBADH Polyclonal Antibody is a Rabbit antibody targeting HIBADH. Anti-HIBADH Polyclonal Antibody can be used in WB.
| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 50 μL | $222 | 7-10 days | 7-10 days | |
| 100 μL | $371 | 7-10 days | 7-10 days | |
| 200 μL | $527 | 7-10 days | 7-10 days |
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In Stock Estimated shipping dateUSA Warehouse[1-2 days] Global Warehouse[5-7 days]
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Product Introduction
Bioactivity
Antigen Details
Chemical Properties
| Description | Anti-HIBADH Polyclonal Antibody is a Rabbit antibody targeting HIBADH. Anti-HIBADH Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| Reactivity | Human,Mouse (predicted:Rat,Cow,Horse,Sheep) |
| Application | |
| Recommended Dose | WB: 1:500-2000 |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Subcellular Localization | Mitochondrion. |
| Tissue Specificity | Detected in skin fibroblasts. |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research 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. |
| Immunogen | KLH conjugated synthetic peptide: human HIBADH |
| Antigen Species | Human |
| Gene Name | HIBADH |
| Gene ID | |
| Protein Name | 3-hydroxyisobutyrate dehydrogenase, mitochondrial |
| Uniprot ID |
| Molecular Weight | Theoretical: 32 kDa. |
Stability & Storage
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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Please see Inhibitor Handling Instructions for more frequently ask questions. Topics include: how to prepare stock solutions, how to store products, and cautions on cell-based assays & animal experiments, etc
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