5-氨基乙酰丙酸合酶1抗体

供货周期： 7天

品牌： Abcam

型号： 0.1ml/100μg

货号：

报价： ¥1

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产品介绍

5-氨基乙酰丙酸合酶1抗体英文名称 Anti-ALAS2/ALAS-E

中文名称 5-氨基乙酰丙酸合酶1抗体

别名 5-aminolevulinate synthase, erythroid-specific, mitochondrial; 5-aminolevulinic acid synthase; ALAS E; ALASE; ANH1; Delta aminolevulinate synthase; XLSA; 5 aminolevulinic acid synthase 2; 5-aminolevulinate synthase 2; 5-aminolevulinate synthase; 5-aminolevulinate synthase 2; Alas 2; ALAS; ALAS E; ALAS, erythroid; ALASE; Aminolevulinate, delta-, synthase 2; Aminolevulinic acid synthase 2, erythroid; ANH1; ASB; Delta ALA synthase 2; Delta ALA synthetase; Delta aminolevulinate synthase 2; Delta aminolevulinate synthase; Erythroid specific ALAS; FLJ93603; XLDPP; XLSA.

浓度 1mg/1ml

规格 0.2ml/200μg

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抗体来源 Rabbit

克隆类型 polyclonal

交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep

产品类型一抗

研究领域肿瘤心血管细胞生物免疫学

蛋白分子量 predicted molecular weight: 59kDa

性状 Lyophilized or Liquid

免疫原 KLH conjugated synthetic peptide derived from human ALAS2/ALAS-E

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.

5-氨基乙酰丙酸合酶1抗体产品介绍 5-aminolevulinate synthase 1 (ALAS-H) and 2 (ALAS-E) are two isoforms of ALAS, an enzyme catalyzing the first step of the heme biosynthetic pathway in mammals. The erythroid-specific isoenzyme, ALAS-E, regulates the first step of hematopoietic cell differentation and iron metabolism in the liver. ALAS-H is a housekeeping protein which mediates synthesis of early heme in the mitochondria of most cells. Succinyl CoA associates with ALAS-E in protein conformation change and translocation of ALAS-E into the mitochondria and does not interact with ALAS-H. The ALAS-E 5'-flanking region contains binding sites for nuclear activators such as GATA-1, NF-E2 and EKLF. Since the ALAS gene maps to the X chromosome, mutation of the gene leads to the pyridoxine-refractory X-linked sideroblastic anemia.

Subunit : Homodimer. Interacts with SUCLA2.

Subcellular Location : Mitochondrion matrix

Tissue Specificity : Erythroid specific.

DISEASE : Defects in ALAS2 are a cause of anemia sideroblastic X-linked (XLSA) [MIM:300751]. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus. XLSA shows a variable hematologic response to pharmacologic doses of pyridoxine.

Defects in ALAS2 are the cause of erythropoietic protoporphyria X-linked dominant (XLDPT) [MIM:300752]. 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. XLDPT is a form of porphyria characterized biochemically by a high proportion of zinc-protoporphyrin in erythrocytes, in which a mismatch between protoporphyrin production and the heme requirement of differentiating erythroid cells leads to overproduction of protoporphyrin in amounts sufficient to cause photosensitivity and liver disease. Note=Gain of function mutations in ALS2 are responsible for XLDPT, but they can also be a possible aggravating factor in congenital erythropoietic porphyria and other erythropoietic disorders caused by mutations in other genes (PubMed:21309041).

Similarity : Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.

Database links : UniProtKB/Swiss-Prot: P22557.2

在纯化抗体时需要控制好几个指标，包括纯度、含量及抗体的抗原结合活性。

纯度：在实验的任何阶段，确定抗体溶液纯度的最简单方法是取一部分样本进行SDS-PAGE电泳。凝胶可用考马斯亮蓝染色(灵敏度为0.1—0.5ug/带)或银染(灵敏度1～l0ug/带)。

定量：如果抗体还不纯，有一个快捷的定量方法，即通过SDS-PAGE电泳分离出轻、重链，然后和已知的标准染色带比较。如果需要分析许多样本，用免疫测定法对抗体定量较容易。如果抗体是经过纯化的，可通过测蛋白总量代替上述两种方法，有一简单的方法，即紫外吸收法。5-氨基乙酰丙酸合酶1抗体的量可通过测280nm处的吸收值来测(10D大致相当于0.75mg/m1的纯化抗体)。

抗原结合活性：一般说来，纯化方法不会引起抗原结合活性的改变。用蛋白G或蛋白A树脂很少导致抗体活性丧失。然而，如果最终抗体产物的作用不如原来所预料的好，检测抗体纯化过程所丢失的活性就极为重要。用一系列滴定法比较纯化的抗体和其原材料的活性，以标定每一步中的总抗体量，这将有助于较好的估计通过纯化所丢失的活性。