HIBADH 抗体 (AA 251-336) (AbBy Fluor® 750)
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北京 101111
Quick Overview for HIBADH 抗体 (AA 251-336) (AbBy Fluor® 750) (ABIN5004614)
抗原
See all HIBADH 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 251-336
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交叉反应
- 人, 小鼠
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预测反应
- Rat,Cow,Sheep,Horse
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human HIBADH
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亚型
- IgG
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anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 1-336) antibody
HIBADH 适用: 人 WB, IF 宿主: 小鼠 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 1-257) antibodyHIBADH 适用: 人, 小鼠, 大鼠 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 128-336) antibodyHIBADH 适用: 人, 小鼠 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 1-336) antibodyHIBADH 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 252-301) antibodyHIBADH 适用: 人, 小鼠, 大鼠, Cow, 犬, 马, 兔, 斑马鱼, 豚鼠, Bat, Hamster, 猴, Pig WB 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (AA 144-193) antibodyHIBADH 适用: 人, 小鼠, 大鼠, Cow, 犬, 马, 兔, Bat, Hamster, 猴, Pig, 非洲爪蟾 WB 宿主: 兔 Polyclonal unconjugated
anti-3-hydroxyisobutyrate Dehydrogenase (HIBADH) (C-Term) antibodyHIBADH 适用: 人, 小鼠, 大鼠, Cow WB, IHC 宿主: 兔 Polyclonal unconjugated
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应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
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别名
- HIBADH
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背景
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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.
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基因ID
- 11112
抗原
-
