HMBS 抗体 (Biotin)
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- 抗原 See all HMBS 抗体
- HMBS (Hydroxymethylbilane Synthase (HMBS))
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适用
- 人, 小鼠, 大鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This HMBS antibody is conjugated to Biotin
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应用范围
- Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
- 交叉反应
- 人, 小鼠, 大鼠
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human HMBS/PBGD
- 亚型
- IgG
- Top Product
- Discover our top product HMBS Primary Antibody
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- 应用备注
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WB 1:300-5000
IHC-P 1:200-400 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C for 12 months.
- 有效期
- 12 months
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- 抗原
- HMBS (Hydroxymethylbilane Synthase (HMBS))
- 别名
- HMBS (HMBS 产品)
- 背景
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Synonyms: HEM3_HUMAN, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase.
Background: PBGD, also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.
- 基因ID
- 3145
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