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HMBS Antibody (PA5-117988) in WB
Western Blot using HMBS Polyclonal Antibody (Product # PA5-117988) at 1:500 dilution. Lane A: HepG2 Whole Cell Lysate, Lane B: HeLa Whole Cell Lysate, Lane C: U937 Whole Cell Lysate, Lane D: K562 Whole Cell Lysate. Lysates/proteins at 30 μg per lane. Secondary antibody: Goat Anti-Rabbit IgG (H+L)/HRP at 1:10,000 dilution. Developed using the ECL technique. Performed under reducing conditions. Predicted band size: 39 kDa. Observed band size: 45 kDa.
产品信息
PA5-117988
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
E. coli-derived Human HMBS fragment
偶联物
形式
Liquid
纯化类型
Antigen affinity chromatography, Protein A
保存液
PBS, pH 7
内含物
0.03% ProClin 300
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2902593
靶标信息
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。
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生物信息学
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; UPS
UniProt ID: (Human) P08397
Entrez Gene ID: (Human) 3145
response to hypoxia
protoporphyrinogen IX biosynthetic process
heme biosynthetic process
response to carbohydrate
response to zinc ion
peptidyl-pyrromethane cofactor linkage
organ regeneration
response to cobalt ion
response to estradiol
response to vitamin
response to drug
response to amino acid
astrocyte differentiation
response to methylmercury
cellular response to antibiotic
cellular response to arsenic-containing substance
cellular response to lead ion
cellular response to cytokine stimulus
cellular response to amine stimulus
cellular response to dexamethasone stimulus
