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RabbitAnti-ALAS-E Rabbit pAb  antibody (bs-9516R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-9516R
英文名称 ALAS-E Rabbit pAb
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.  
研究领域 肿瘤  心血管  细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 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.
理论分子量 59 kDa
检测分子量
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ALAS2/ALAS-E: 101-200/587 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
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.

SWISS:
P22557

Gene ID:
212

Database links:

Entrez Gene: 212 Human

Entrez Gene: 11656 Mouse

Entrez Gene: 25748 Rat

Omim: 301300 Human

SwissProt: P22557 Human

SwissProt: P08680 Mouse

SwissProt: Q63147 Rat

Unigene: 522666 Human

Unigene: 555936 Human

Unigene: 302724 Mouse

Unigene: 226279 Rat

Unigene: 32517 Rat



产品图片
Sample: Lane 1: Human MOLT4 cell lysates Lane 2: Human K562 cell lysates Primary: Anti-ALAS-E (bs-9516R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 59 kDa Observed band size: 62 kDa
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