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Anti-ALAS1/ALAS-H5-氨基乙酰丙酸合酶1抗体
点击次数：261发布时间：2012/12/26 2:48:54
更新日期：2024/9/5 14:43:27
所 在 地：其它
产品型号：BY-9527R
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货号：BY-9527R
英文名称：Anti-ALAS1/ALAS-H
中文名称：5-氨基乙酰丙酸合酶1抗体
其他名称：mitochondrial; nonspecific;5 aminolevulinate synthase;5 aminolevulinate synthase nonspecific mitochondrial;5 aminolevulinic acid synthase;5-aminolevulinate synthase;5-aminolevulinic acid synthase 1;Alas 1;ALAS 3;ALAS;ALAS H;ALAS HOUSEKEEPING TYPE;ALAS N;ALAS-H;alaS1;ALAS3;ALASH;Aminolevulinate delta synthase 1;Aminolevulinic acid synthase 1;Delta ALA synthetase;Delta aminolevulinate synthase;Delta-ALA synthase 1;Delta-aminolevulinate synthase 1;HEM1_HUMAN;MIG 4;MIG4;Migration inducing protein 4.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 70kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, rat, mo, Horse, cow
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.Subcellular Location : Mitochondrion matrix
