细胞色素b5还原酶3抗体

我公司专业供应细胞色素b5还原酶3抗体。了解更多关于的细胞色素b5还原酶3抗体英文名称细胞色素b5还原酶3抗体产品别名规格请看以下说明书。

英文名称  Anti-CYB5R3     

中文名称  细胞色素b5还原酶3抗体     

别    名  B5R; Cyb5r3; Cytochrome b5 reductase 3; Cytochrome b5 reductase; DIA1; Diaphorase 1; Diaphorase-1; NADH cytochrome b5 reductase 3; NADH-cytochrome b5 reductase 3 membrane-bound form; NADH-cytochrome b5 reductase 3 soluble form; NB5R3_HUMAN; OTTHUMP00000028761; OTTHUMP00000198435; OTTHUMP00000198574; OTTHUMP00000198662; OTTHUMP00000198665.     

浓    度  1mg/1ml     

规 格  0.2ml/200μg     

细胞色素b5还原酶3抗体的功能：

抗体的主要功能是与抗原（包括外来的和自身的）相结合，从而有效地清除侵入机体内的微生物、寄生虫等异物，抗体（antibody）是一种应答抗原产生的、可与抗原特异性结合的蛋白质。每种抗体与特定的抗原决定基结合。这种结合可以使抗原失活，也可能无效但有时也会对机体造成病理性损害，如抗核抗体、抗双链DNA抗体、抗甲状腺球蛋白抗体等一些自身抗体的产生，对人体可造成危害。

抗体来源  Rabbit  

克隆类型  polyclonal

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

产品类型  一抗    

研究领域  心血管 细胞生物 信号转导 脂蛋白 新陈代谢  

蛋白分子量  predicted molecular weight: 34kDa

性    状  Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human CYB5R3

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  ICC=1:100-500  IF=1:100-500

（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 CYB5R3 is a 301 amino acid protein encoded by the human gene CYB5R3. CYB5R3 belongs to the flavoprotein pyridine nucleotide cytochrome reductase family and has two naturally occuring isoforms. Isoform 1 is anchored to the cytoplasmic side of the endoplasmic reticulum membrane and mitochondrion outer membrane, while isoform 2 is the soluble form found in erythrocytes. CYB5R3 is involved in the desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism and, in erythrocytes, methemoglobin reduction. A serine residue at position 117 seems to only be found in persons of African origin. The allele frequency is 0.23 in African Americans. It is not found in Caucasians, Asians, Indo-Aryans or Arabs. This difference seems to have no effect on the enzyme activity. Defects in CYB5R3 are the cause of hereditary methemoglobinemia (HM). There are three forms of this disease: type 1 (HM1), in which the enzyme is only deficient in erythrocytes with a mild cyanosis; type 2 (HM2), in which the enzyme is completely deficient; and type 3 (HM3), where the deficiency is seen in all blood cells. Type 2 is a severe form accompanied by mental retardation and neurological impairment.

Function : Desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism, and, in erythrocyte, methemoglobin reduction.

Subunit : Component of a complex composed of cytochrome b5, NADH-cytochrome b5 reductase (CYB5R3) and MOSC2 (By similarity).

Subcellular Location : Endoplasmic reticulum membrane. Mitochondrion outer membrane and Cytoplasm. Produces the soluble form found in erythrocytes.

Tissue Specificity : Isoform 2 is expressed at late stages of erythroid maturation.

DISEASE : Defects in CYB5R3 are the cause of methemoglobinemia CYB5R3-related (METHB-CYB5R3) [MIM:250800]. A form of methemoglobinemia, a hematologic disease characterized by the presence of excessive amounts of methemoglobin in blood cells, resulting in decreased oxygen carrying capacity of the blood, cyanosis and hypoxia. There are two types of methemoglobinemia CYB5R3-related. In type 1, the defect affects the soluble form of the enzyme, is restricted to red blood cells, and causes well-tolerated methemoglobinemia. In type 2, the defect affects both the soluble and microsomal forms of the enzyme and is thus generalized, affecting red cells, leukocytes and all body tissues. Type 2 methemoglobinemia is associated with mental deficiency and other neurologic symptoms.

细胞色素b5还原酶3抗体Similarity : Belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. Contains 1 FAD-binding FR-type domain.

Database links : UniProtKB/Swiss-Prot: P00387.3