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細胞色素b5還原酶3抗體
  • 產品貨號：
    BN40354R
  • 中文名稱：
    細胞色素b5還原酶3抗體
  • 英文名稱：
    Rabbit anti-CYB5R3 Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產品規(guī)格

    售價

    備注

  • BN40354R-100ul

    100ul

    ￥2360.00

    交叉反應：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse,Sheep) 推薦應用：WB,IHC-P,ELISA

  • BN40354R-200ul

    200ul

    ￥3490.00

    交叉反應：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse,Sheep) 推薦應用：WB,IHC-P,ELISA

產品描述

英文名稱CYB5R3
中文名稱細胞色素b5還原酶3抗體
別    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.  
研究領域心血管  細胞生物  信號轉導  脂蛋白  新陳代謝  
抗體來源Rabbit
克隆類型Polyclonal
交叉反應Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產品應用WB=1:500-2000 ELISA=1:5000-10000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量34kDa
細胞定位細胞漿 細胞膜 
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human CYB5R3:101-200/301 
亞    型IgG
純化方法affinity purified by Protein A
儲 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產品介紹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.

Similarity:
Belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. Contains 1 FAD-binding FR-type domain.

SWISS:
P00387

Gene ID:
1727

Database links:

Entrez Gene: 1727 Human

Entrez Gene: 25035 Rat

Omim: 613213 Human

SwissProt: P00387 Human

SwissProt: P20070 Rat

Unigene: 561064 Human

Unigene: 35994 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.


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