货号 |
bs-12162R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-CYB5R3 antibody |
中文名称 |
Rabbit Anti-CYB5R3 antibody |
研究领域 |
心血管,细胞生物,信号转导,脂蛋白,新陈代谢, |
英文别名 |
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; OTTHUMP0000002 |
反应物种(已验证) |
Human,Mouse,Rat |
反应物种(预测) |
Chicken,Dog,Pig,Cow,Horse,Sheep |
产品应用(已验证) |
WB,IHC |
产品应用(可推荐) |
ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000,IHC-P=1:100-500, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
34 |
细胞定位 |
细胞浆,细胞膜 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human CYB5R3 |
抗原表位 |
101-200/301 |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Endoplasmic reticulum membrane. Mitochondrion outer membrane and Cytoplasm. Produces the soluble form found in erythrocytes. |
Tissue |
Isoform 2 is expressed at late stages of erythroid maturation. |
SIMILARITY |
Belongs to the flavoprotein pyridine nucleotide cytochrome reductase family.
Contains 1 FAD-binding FR-type domain. |
SUBUNIT |
Component of a complex composed of cytochrome b5, NADH-cytochrome b5 reductase (CYB5R3) and MOSC2 (By similarity). |
Function |
Desaturation and elongation of fatty acids, cholesterol biosynthesis, drug metabolism, and, in erythrocyte, methemoglobin reduction. |
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 |
SWISS |
P00387 |
Gene ID |
1727 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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. |