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| 应用 | 建议稀释比 | 已发表文章 |
|---|---|---|
免疫印迹 (WB) |
1:500-1:2,000 | - |
免疫组化（石蜡） (IHC (P)) |
1:50-1:200 | - |
| 产品规格 | |
|---|---|
种属反应 |
Human, Mouse |
宿主/亚型 |
Rabbit / IgG |
分类 |
Polyclonal |
类型 |
Antibody |
抗原 |
Recombinant full length human HMBS |
偶联物 |
Unconjugated |
形式 |
Liquid |
浓度 |
1 mg/mL |
纯化类型 |
Antigen affinity chromatography |
保存液 |
PBS with 50% glycerol |
内含物 |
0.02% sodium azide |
保存条件 |
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. |
RRID |
AB_2554031 |
This antibody detects endogenous protein at a molecular weight of 39 kDa.
Purity is >95% by SDS-PAGE.
PBGD (porphobilinogen deaminase), 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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: HEM3; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; Porphobilinogen deaminase; porphyria, acute; Chester type; Pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase
基因别名: HMBS; PBG-D; PBGD; PORC; T25658; UPS; Uros1
UniProt ID: (Human) P08397, (Mouse) P22907
Entrez Gene ID: (Human) 3145, (Mouse) 15288
分子生物学功能:
deaminase
