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产品信息
PA5-52253
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
偶联物
形式
Liquid
浓度
0.3 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.2, with 40% 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.
运输条件
Wet ice
RRID
AB_2642470
产品详细信息
Immunogen sequence: QFEIIAMSTT GDKILDTALS KIGEKSLFTK ELEHALEKNE VDLVVHSLKD LPTVLPPGFT IGAICKRENP HDAVVFHPKF VGKTLETLPE KSVVGTSSLR RAAQLQRKFP HLEFRSIRGN LNTRL
Highest antigen sequence identity to the following orthologs: Mouse - 94%, Rat - 94%.
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: alternative name: porphobilinogen deaminase; 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: hemC; HMBS; PBG-D; PBGD; PORC; T25658; UPS; URO-S; Uros1
UniProt ID: (Human) P08397, (Mouse) P22907
Entrez Gene ID: (Human) 3145, (Mouse) 15288, (Rat) 25709
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
porphyrin-containing compound biosynthetic process
tetrapyrrole biosynthetic process
response to hormone
response to metal ion
response to lead ion
response to organic cyclic compound
response to nutrient levels
