供货周期: | 一周 |
品牌: | EterLife |
规格: | 0.1ml/100μg 、0.2ml/200μg |
货号: | TE-KT-5114 |
CAS号: | 详见产品说明书 |
卟胆原脱氨酶抗体
〖别名〗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.
〖浓度〗1mg/1ml
〖规格〗0.2ml/200μg
〖抗体来源〗Rabbit
〖克隆类型〗polyclonal
〖交叉反应〗卟胆原脱氨酶抗体Human, Mouse, Rat, Chicken, Pig, Cow, Rabbit, Sheep, Cat
〖产品类型〗一抗
〖研究领域〗肿瘤 细胞生物 免疫学 信号转导
〖蛋白分子量〗predicted molecular weight: 39kDa
〖性状〗Lyophilized or Liquid
〖免疫原〗KLH conjugated synthetic peptide derived from human HMBS/PBGD
〖亚型〗IgG
〖纯化方法〗affinity purified by Protein A
〖储存液〗Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
〖产品应用〗WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200
(石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
〖保存条件〗Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
〖产品介绍〗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.
Function : Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
Subcellular Location : Cytoplasm
Tissue Specificity : Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
DISEASE : Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
Similarity : Belongs to the HMBS family.
Database links :
Entrez Gene: 3145 Human
Entrez Gene: 15288 Mouse
Entrez Gene: 396581 Pig
Entrez Gene: 25709 Rat
Omim: 609806 Human
SwissProt: P08397 Human
SwissProt: P22907 Mouse
SwissProt: P19356 Rat
Unigene: 82609 Human
Unigene: 247676 Mouse
Unigene: 11080 Rat
卟胆原脱氨酶,为血红素等四吡咯环化合物合成通路中的催化酶。PBGD催化卟胆原(Porphobilingen,PBG)底物的线性四聚化,反应过程中四分子卟胆原底物有序性的共价连接到酶的辅基上,最后水解形成具有四联吡咯环结构的尿卟啉原前体。PBGD的活力缺陷会引起急性间歇性卟啉症(Acute Intermittant Porphyria,AIP)的发生。
做过实验的亲们都知道卟胆原脱氨酶抗体反应规律,仔细观察我们会发现当抗原第一次进入机体时,需经一定的潜伏期才能产生抗体,且抗体产生的量也不多,在体内维持的时间也较短。当相同抗原第二次进入机体后,开始时,由于原有抗体中的一部分与再次进入的抗原结合,可使原有抗体量略为降低。随后,抗体效价迅速大量增加,可比初次反应产生的多几倍到几十倍,在体内留存的时间亦较长。因此得出由抗原刺激机体产生的抗体,经过一定时间后可逐渐消失。此时若再次接触抗原,可使已消失的抗体快速上升。如再次刺激机体的抗原与初次相同,则称为特异性回忆反应;若与初次反应不同,则称为非特异性回忆反应。
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Ab-(010)-17313 Nm23(NDP Kinase A,NDPKA) 肿瘤转移抑制基因抗原 ,规格: 0.5mg ,英文名: Nm23
Ab-(010)-17314 NKG2D(natural killer cell group 2D) NK细胞受体/自然杀伤细胞活化性受体 ,规格: 0.5mg ,英文名: NKG2D
Ab-(010)-17315 NKG2A/CD159a/KLRC1 NK细胞受体2A/自然杀伤细胞活化性受体2A抗原 ,规格: 0.5mg ,英文名: NKG2A/CD159a/KLRC1
Ab-(010)-17316 NKA(Neurokinin A) 神经激肽A抗原 ,规格: 0.5mg ,英文名: NKA
Ab-(010)-17317 NIT2 (nitrilase family, member 2) NIT2蛋白抗原 ,规格: 0.5mg ,英文名: NIT2
Ab-(010)-17318 NGX6 (nasopharyngeal carcinoma/NPC associated gene 6) 鼻咽癌细胞相关基因6(多肽) ,规格: 0.5mg ,英文名: NGX6
Ab-(010)-17319 NGN3(neurogenin 3; Neurog3) 神经元素3抗原 ,规格: 0.5mg ,英文名: NGN3
Ab-(010)-17320 NF-κBp50(p50 NF-kappa B;p50NFKB) 细胞核因子50/κ基因结合核因子50抗原 ,规格: 0.5mg ,英文名: NF-κBp50
Ab-(010)-17321 phospho-NFKB p65 (pSer536) peptide 磷酸化细胞核因子/磷酸化k基因结合核因子抗原 ,规格: 0.5mg ,英文名: phospho-NFKB
Ab-(010)-17322 NFATc1 活化T细胞核因子1蛋白 ,规格: 0.5mg ,英文名: NFATc1
Ab-(010)-17323 NF2/merlin(neurofibromatosis type 2) 2型神经纤维瘤抗原 ,规格: 0.5mg ,英文名: NF2/merlin
Ab-(010)-17324 Neurocan 神经粘蛋白抗原 ,规格: 0.5mg ,英文名: Neurocan
Ab-(010)-17325 Nephrin Protein 去氧肾上腺素 ,规格: 0.5mg ,英文名: Nephrin
Ab-(010)-17326 E-cadherin E-钙粘附分子抗原 ,规格: 0.5mg ,英文名: E-cadherin
Ab-(010)-17327 N-cadherin N-钙粘附分子抗原 ,规格: 0.5mg ,英文名: N-cadherin
Ab-(010)-17328 NAP1 (nucleosome assembly protein 1) 核小体组装蛋白1(多肽) ,规格: 0.5mg ,英文名: NAP1
Ab-(010)-17329 Natrexone/BSA:Natrexone/KLH 纳曲酮偶联牛血清白蛋白:纳曲酮偶联血蓝蛋白 ,规格: 0.5mg ,英文名: Natrexone/BSA:Natrexone/KLH
Ab-(010)-17330 Nanog fragment 干细胞关键蛋白(多肽) ,规格: 0.5mg ,英文名: Nanog
Ab-(010)-17331 CHRNA7(Nicotinic-Acetylcholine receptor alpha 7) 烟碱型乙酰胆碱受体α7(多肽) ,规格: 0.5mg ,英文名: CHRNA7
Ab-(010)-17332 MYOZ/MYOZ1(Myozenin)human、ret、mouse MYOZ抗原 ,规格: 0.5mg ,英文名: MYOZ/MYOZ1
Ab-(010)-17333 MYSM1(Myb-like, SWIRM and MPN domain-containing protein 1) MYSM1抗原 ,规格: 0.5mg ,英文名: MYSM1
Ab-(010)-17334 PMP2/FABP8(peripheral myelin protein 2) 周围神经髓鞘蛋白2抗原 ,规格: 0.5mg ,英文名: PMP2/FABP8
Ab-(010)-17335 MyD88 (myeloid differential protein-88) 髓样分化蛋白抗原 ,规格: 0.5mg ,英文名: MyD88
Ab-(010)-17336 Muc-1/CD227 Anti-gen (mucin-1) 粘蛋白-1/上皮膜抗原 ,规格: 0.5mg ,英文名: Muc-1/CD227
Ab-(010)-17337 mTOR(Mammalian target of rapamycin) 雷帕霉素靶蛋白 ,规格: 0.5mg ,英文名: mTOR
Ab-(010)-17338 MLN(Motilin)peptide 胃动素多肽 ,规格: 0.5mg ,英文名: MLN
Ab-(010)-17339 MTNR1B/MTNR-1B(Melatonin receptor 1B) 褪黑素受体1B抗原 ,规格: 0.5mg ,英文名: MTNR1B/MTNR-1B
Ab-(010)-17340 MUC5AC/Mucin 5AC(Gastric Mucin M1) 胃粘液素抗原 ,规格: 0.5mg ,英文名: MUC5AC/Mucin
Ab-(010)-17341 MLH1(Mutl homolog l gene) 错配修复蛋白1抗原 ,规格: 0.5mg ,英文名: MLH1
Ab-(010)-17342 MrpL28 (mitochondrial ribosomal protein L28) 线粒体核糖体蛋白L28(抗原) ,规格: 0.5mg ,英文名: MrpL28
Ab-(010)-17343 MRP5/ABCC5(Multidrug Resistanec-Associated Protein 5) 多药耐药相关蛋白5抗原 ,规格: 0.5mg ,英文名: MRP5/ABCC5
Ab-(010)-17344 MRP2 (multidrug resistance-associated protein2) 多药耐药相关蛋白2 ,规格: 0.5mg ,英文名: MRP2
Ab-(010)-17345 MPO peptide 髓过氧化物酶抗原 ,规格: 0.5mg ,英文名: MPO
Ab-(010)-17346 MMP-10(matrix metalloproteinase 10) 基质金属蛋白酶-10抗原 ,规格: 0.5mg ,英文名: MMP-10
Ab-(010)-17347 MMP-9(matrix metalloproteinase 9) 基质金属蛋白酶-9抗原 ,规格: 0.5mg ,英文名: MMP-9
Ab-(010)-17348 MMP-26(Matrix metalloproteinase-26) 基质金属蛋白酶-26抗原 ,规格: 0.5mg ,英文名: MMP-26
Ab-(010)-17349 MMP-17(Matrix metalloproteinase-17) 基质金属蛋白酶-17抗原 ,规格: 0.5mg ,英文名: MMP-17
Ab-(010)-17350 MMP-20(matrix metalloproteinase 20) 基质金属蛋白酶20抗原 ,规格: 0.5mg ,英文名: MMP-20
Ab-(010)-17351 MMP-13 (Matrix metalloproteinase 13) 基质金属蛋白酶13抗原 ,规格: 0.5mg ,英文名: MMP-13
Ab-(010)-17352 MIP2 (myocardial ischemic preconditioning upregulated protein 2) 巨噬细胞炎症蛋白2抗原 ,规格: 0.5mg ,英文名: MIP2
Ab-(010)-17353 CD122/IL-2RB(Imterleukm-2 Receptor beta) CD122/白介素2受体β链抗原 ,规格: 0.5mg ,卟胆原脱氨酶抗体英文名: CD122/IL-2RB
Ab-(010)-17354 MIP-1 Beta (macrophage inflammatory protein 1 Beta) 巨噬细胞炎症因子1β 抗原 ,规格: 0.5mg ,英文名: MIP-1
Ab-(010)-17355 MIF (Macrophage Migration Inhibitory Factor) 巨噬细胞移动抑制因子抗原 ,规格: 0.5mg ,英文名: MIF
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